Emergencies in haematology

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Emergencies in

Emergencies in

hematology

hematology

M. Zawartko, MD

M. Zawartko, MD

Department of Hematology,

Department of Hematology,

Pomeranian Medical University,

Pomeranian Medical University,

Szczecin

Szczecin

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Emergencies in hematology

Emergencies in hematology

ATLS

ATLS

Hypercalcemia

Hypercalcemia

Spinal cord compression

Spinal cord compression

Superior vena cava syndrome

Superior vena cava syndrome

Leukocytostasis

Leukocytostasis

Hyperviscosity

Hyperviscosity

Pericardial effusion/tamponade

Pericardial effusion/tamponade

Intestinal obstruction

Intestinal obstruction

Urinary obstruction

Urinary obstruction

Malignant biliary obstruction

Malignant biliary obstruction

Increased intracranial pressure

Increased intracranial pressure

Neoplastic menigitis

Neoplastic menigitis

Other….

Other….

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ATLS – Acute tumor lysis

ATLS – Acute tumor lysis

syndrome

syndrome

Is characterized by various combinations of:

Is characterized by various combinations of:

-

hyperuricemia;

hyperuricemia;

-

Hypergl

Hypergl

y

y

cemia;

cemia;

-

Hyperkalemia;

Hyperkalemia;

-

Hyperphosphatemia;

Hyperphosphatemia;

-

Hypocalcemia;

Hypocalcemia;

-

Lactic acidosis.

Lactic acidosis.

Is caused by the destruction of a large

Is caused by the destruction of a large

number of rapidly proliferating neoplastic

number of rapidly proliferating neoplastic

cells.

cells.

Frequently: acute renal failure develops as a

Frequently: acute renal failure develops as a

result of the syndrome.

result of the syndrome.

-Uric acid
-Glucose
-Potassium
-Phosphate

s
-Lactic acid

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ATLS – Acute tumor lysis

ATLS – Acute tumor lysis

syndrome

syndrome

Most common offenders:

Leukemia

ALL & AML

Lymphoma

Aggressive or bulky

Also described in

MM, SCLC, breast, ovarian

ATLS usually occurs during or shortly (1-5 days)

ATLS usually occurs during or shortly (1-5 days)

after chemotherapy;

after chemotherapy;

In rare cases – spontaneous necrosis of

In rare cases – spontaneous necrosis of

malignancies causes ATLS – prior to chemotherapy.

malignancies causes ATLS – prior to chemotherapy.

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ATLS – Acute tumor lysis

ATLS – Acute tumor lysis

syndrome

syndrome

Hyperuricemia:

Hyperuricemia:

May be present at the time of chemotherapy

May be present at the time of chemotherapy

Effective treatment kills malignant cells and

Effective treatment kills malignant cells and

leads to increased serum uric acid levels

leads to increased serum uric acid levels

from the turnover of nucleic acids.

from the turnover of nucleic acids.

Uric acid can precipitate in the tubules,

Uric acid can precipitate in the tubules,

medulla and collecting ducts of the kidney

medulla and collecting ducts of the kidney

leading to renal failure.

leading to renal failure.

Lactic acidosis and dehydratation may

Lactic acidosis and dehydratation may

contribute to the precipitation of uric acid in

contribute to the precipitation of uric acid in

the renal tubules.

the renal tubules.

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ATLS – Acute tumor lysis

ATLS – Acute tumor lysis

syndrome

syndrome

Hyperphosphatemia

Hyperphosphatemia

Produces reciprocal depression in serum calcium

Produces reciprocal depression in serum calcium

Hypocalcemia – symptoms: neuromuscular irritability,

Hypocalcemia – symptoms: neuromuscular irritability,

tetany;

tetany;

Deposition of calcium phosphate in the kidney and

Deposition of calcium phosphate in the kidney and

hyperphosphatemia may cause renal failure.

hyperphosphatemia may cause renal failure.

Hyperkalemia

Hyperkalemia

-

Potassium is the principal intracellular cation and

Potassium is the principal intracellular cation and

massive destruction of malignant cells may lead to

massive destruction of malignant cells may lead to

hyperkalemia.

hyperkalemia.

-

In patients with renal failure may rapidly be life –

In patients with renal failure may rapidly be life –

threatening.

threatening.

-

Can cause ventricular arrythmias and sudden death.

Can cause ventricular arrythmias and sudden death.

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ATLS – Acute tumor lysis

ATLS – Acute tumor lysis

syndrome

syndrome

The most important predictive factors:

The most important predictive factors:

-

tumor burden: hyperuricemia and high

tumor burden: hyperuricemia and high

levels of lactate dehydrogenase

levels of lactate dehydrogenase

(LDH>1500 U/l);

(LDH>1500 U/l);

-

Renal function

Renal function

Recognition & prevention are the most

Recognition & prevention are the most

important steps in the management of

important steps in the management of

this syndrome.

this syndrome.

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ATLS – Acute tumor lysis

ATLS – Acute tumor lysis

syndrome

syndrome

Prophylaxis

Prophylaxis

Maintain hydratation by administration of ivf

Maintain hydratation by administration of ivf

at 3000 ml/m2 per day

at 3000 ml/m2 per day

Keep urine pH at 7,0 or greater by

Keep urine pH at 7,0 or greater by

administration of sodium bicarbonate

administration of sodium bicarbonate

Administer allopurinol at 300 mg/m2 per day

Administer allopurinol at 300 mg/m2 per day

Monitor serum chemistry.

Monitor serum chemistry.

Start chemotherapy when serum uric acid <8,0

Start chemotherapy when serum uric acid <8,0

mg/dl; serum creatinine <1,6 mg/dl; urine pH

mg/dl; serum creatinine <1,6 mg/dl; urine pH

>7,0.

>7,0.

If an urgent anticancer therapy is needed

If an urgent anticancer therapy is needed

consider dialysis.

consider dialysis.

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ATLS – Acute tumor lysis

ATLS – Acute tumor lysis

syndrome

syndrome

In some cases, uric acid levels cannot be lowered sufficiently with the standard preventive

approach.

Rasburicase

can be effective in these instances.

Rasburicase

Recombinant urate oxidase enzyme

In humans, gene for this is inactivated

Converts uric acid to allantoin

Acts rapidly, decreasing uric acid levels within hours

Xanthine

Xanthine

Xanthin
e
oxidase

Uric Acid

Uric Acid

Allantoin

Allantoin

Urate Oxidase

Allopurin
ol

Hypoxanthi

Hypoxanthi

ne

ne

Xanthine
oxidase

Rasburicase

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ATLS – Acute tumor lysis

ATLS – Acute tumor lysis

syndrome

syndrome

Despite aggressive

Despite aggressive

prophylaxis ATLS

prophylaxis ATLS

and/or oliguric or

and/or oliguric or

anuric renal failure

anuric renal failure

may occur;

may occur;

Dialysis is often

Dialysis is often

necessary and

necessary and

should be considered

should be considered

early in the course.

early in the course.

The prognosis is

The prognosis is

good, and renal

good, and renal

function recovers

function recovers

after the uric acid

after the uric acid

level is lowered to <

level is lowered to <

10 mg/dl.

10 mg/dl.

If:

If:

Serum K+ > 6mEq/l

Serum K+ > 6mEq/l

Serum uric acid >10

Serum uric acid >10

mg/dl

mg/dl

Serum

Serum

creatinine>10 mg/dl

creatinine>10 mg/dl

Serum

Serum

phosphate>10mg/dl

phosphate>10mg/dl

or increasing

or increasing

symptomatic

symptomatic

hypocalcemia

hypocalcemia

present

present

Treatment

Treatment

Begin

Begin

hemodialysis

hemodialysis

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Hypercalcemia

Hypercalcemia is the most common life-threatening metabolic

Hypercalcemia is the most common life-threatening metabolic

disorder associated with neoplastic diseases, occurring in an

disorder associated with neoplastic diseases, occurring in an

estimated 10% to 20% of all adults with cancer. It also occurs in

estimated 10% to 20% of all adults with cancer. It also occurs in

children with cancer, but with much less frequency

children with cancer, but with much less frequency

(approximately 0.5%-1%).

(approximately 0.5%-1%).

Causes

Causes

The main causes of hypercalcemia due to cancer are an increase

The main causes of hypercalcemia due to cancer are an increase

in the amount of calcium absorbed from the bones, and an

in the amount of calcium absorbed from the bones, and an

inability of the kidneys to excrete excess calcium. Some cancer

inability of the kidneys to excrete excess calcium. Some cancer

cells secrete substances that cause calcium to be absorbed into

cells secrete substances that cause calcium to be absorbed into

the bloodstream from bones. Immobility, dehydration, anorexia,

the bloodstream from bones. Immobility, dehydration, anorexia,

nausea, and vomiting may also increase calcium levels.

nausea, and vomiting may also increase calcium levels.

Incidence

Incidence

Hypercalcemia occurs most frequently in patients with lung and

Hypercalcemia occurs most frequently in patients with lung and

breast cancer. It may also occur in patients with multiple

breast cancer. It may also occur in patients with multiple

myeloma, head and neck cancer, cancer of unknown primary

myeloma, head and neck cancer, cancer of unknown primary

origin, lymphoma, leukemia, kidney cancer, and

origin, lymphoma, leukemia, kidney cancer, and

gastrointestinal cancer.

gastrointestinal cancer.

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Hypercalcemia

• History: Advanced

malignancy, especially:

multiple myeloma, breast

cancer, non-small cell lung

cancer

• Symptoms:
- Polydipsia, polyuria
- Anorexia
- Nausea, vomiting
- Lethargy, drowsiness
- Constipation, obstipation

• Signs:
- Dehydration, hypotension
- Hyporefllexia, muscle

weakness, confusion,

seizure, coma

- Ileus

• ECG
- Bradycardia
- Shortened P-R interval
- Shortened Q-T interval
- Wide T-waves
- Supraventrial and ventricular

arrhythmia

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Hypercalcemia

Symptoms may

Symptoms may

be classified by the affected body

be classified by the affected body

part:

part:

Nervous system

Nervous system

Calcium plays a major role in the normal functioning of

Calcium plays a major role in the normal functioning of

the central nervous system (the brain and spinal cord).

the central nervous system (the brain and spinal cord).

Symptoms of hypercalcemia may include weakness,

Symptoms of hypercalcemia may include weakness,

loss of reflexes in the muscles, and decreased stamina.

loss of reflexes in the muscles, and decreased stamina.

Patients with central nervous system symptoms may

Patients with central nervous system symptoms may

have changes in personality, difficulty thinking or

have changes in personality, difficulty thinking or

speaking clearly, disorientation, or hallucinations.

speaking clearly, disorientation, or hallucinations.

Eventually, coma may result. Headaches can also occur,

Eventually, coma may result. Headaches can also occur,

which can be made worse by vomiting and dehydration.

which can be made worse by vomiting and dehydration.

Heart

Heart

Hypercalcemia affects normal heart rhythms and

Hypercalcemia affects normal heart rhythms and

increases sensitivity to some heart medications (such

increases sensitivity to some heart medications (such

as digoxin). As calcium levels increase, irregular

as digoxin). As calcium levels increase, irregular

heartbeats may develop, and may lead to a heart

heartbeats may develop, and may lead to a heart

attack.

attack.

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Hypercalcemia

Symptoms may

Symptoms may

be classified by the affected body

be classified by the affected body

part:

part:

Gastrointestinal

Gastrointestinal

Increased stomach acid often is produced with hypercalcemia

Increased stomach acid often is produced with hypercalcemia

and may intensify loss of appetite, nausea, and vomiting.

and may intensify loss of appetite, nausea, and vomiting.

Constipation may result from the dehydration associated

Constipation may result from the dehydration associated

with hypercalcemia.

with hypercalcemia.

Kidney

Kidney

Hypercalcemia causes the kidneys to not function correctly,

Hypercalcemia causes the kidneys to not function correctly,

leading to the production of large volumes of urine. The

leading to the production of large volumes of urine. The

large amount of urine combined with less liquid intake leads

large amount of urine combined with less liquid intake leads

to symptoms of dehydration, including thirst, dry mouth,

to symptoms of dehydration, including thirst, dry mouth,

little or no sweating, and concentrated urine. Patients with

little or no sweating, and concentrated urine. Patients with

myeloma often have kidney problems due to hypercalcemia.

myeloma often have kidney problems due to hypercalcemia.

Kidney stones may result from long-term hypercalcemia.

Kidney stones may result from long-term hypercalcemia.

Bone

Bone

Hypercalcemia of cancer can result from bone metastases or

Hypercalcemia of cancer can result from bone metastases or

bone loss, and may contribute to broken bones, bone

bone loss, and may contribute to broken bones, bone

disfigurement, and pain.

disfigurement, and pain.

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Hypercalcemia of Malignancy

Suspect hypercalcemia

Diagnosis

Diagnosis

-Measure blood pressure and heart rate lying and standing (if

possible)

-ECG

-Measure serum calcium, phosphate, albumin, urea, creatinine

-Correct serum calcium

Corrected Ca = measured Ca(mmol/L) + [40 - serum albumin (g/L)] x

0.027

Determine urgency of

treatment

Outpatient-based treatment

Outpatient-based treatment
-Serum calcium < 3.0 mmol/L
-Alert and oriented
-No significant nausea
-Adequate intravascular
volume
-Normal renal function
-No significant ECG
abnormality
-mild constipation

Hospital-based treatment

Hospital-based treatment
-Serum calcium > 3.0 mmol/L
-Altered level of consciousness
-Nausea or vomiting
-Intravascular volume
contraction
-Renal Dysfunction
-Significant ECG abnormality
-obstipation, ileus

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Hypercalcemia of Malignancy

Treatment

(treat underlying malignancy when

(treat underlying malignancy when

possible)

possible)

General Measures

General Measures

-Avoid immobilization (if possible)

-Discontinue calcium supplements, vitamin D, cimetidine, NSAIDs,

thiazides

Hospital -based treatment

Hospital -based treatment
- Replacing fluids is the first and most
important step in treating moderate or
severe hypercalcemia.
-Use furosemide 20-40 mg IV if concerns
of volume overload
-Drugs that may help stop the
breakdown of bone include calcitonin,
plicamycin (mithramycin),
bisphosphonates (etidronate,
pamidronate, and clodronate), and
gallium nitrate.

-Steroids and phosphate may also be
used to treat hypercalcemia.

-Dialysis is used in patients with kidney
failure.
-Combinations of drugs may also be
used.

Outpatient-based

Outpatient-based

treatment

treatment
-Maintain adequate oral fluid
intake
-Correct serum phosphate
with oral supplements
-For breast cancer,
lymphomas or myelomas
give prednisone 40-100 mg
PO daily
-Arrange for urgent follow-up
with oncologist

1.

http://www.bccancer.bc.ca/HPI/Chemotherap
yProtocols/SupportiveCare/default.htm

2.Cancer: Principles and Practice of Oncology

(4th ed.) 1997: DeVita S, Hellman S,
Rosenberg S,

ed.. Vol. 2, Section 3 Metabolic

Emergencies:Hypercalcemia, pages 2486-
2493.

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Superior vena cava syndrome

Superior vena cava syndrome

(SVCS)

(SVCS)

The superior vena

The superior vena

cava syndrome is

cava syndrome is

the group of

the group of

symptoms that

symptoms that

result from

result from

compression of the

compression of the

large vein (superior

large vein (superior

vena cava) that

vena cava) that

transmits blood to

transmits blood to

the heart from the

the heart from the

head, neck and

head, neck and

upper extremities.

upper extremities.

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Superior vena cava

Superior vena cava

syndrome

syndrome

SVCS is the clinical manifestation of the SVC

obstruction

– External (mass effect)
– Internal (thrombus)
Etiology

Malignancy (78-85%)

- Lung (small-cell and squamous-cell histologies,

accounts for ~85% off all cases of malignant origin)

- Lymphoma =>

YOUNG ADULTS!!

YOUNG ADULTS!!

- Others (primary mediastinal germ cell, thymoma)

Infection

Thrombosis

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Superior vena cava

Superior vena cava

syndrome

syndrome

Up to 60% with SVCS will not have

prior cancer diagnosis

Symptoms & signs

-

Dyspnea

-

Caugh

-

Neck & facial swelling (especially

around the eyes) (+/- plethora)

-

Venous distention

-

Other: hoarseness, tongue swelling,

headaches, nasal congestion,

epistaxis, hemopthysis, dysphagia,

pain, dizziness, syncope, and letargy.

-

Dilated veins

-

Increased number of collateral veins

covering the anterior chest wall

-

Cyanosis

-

Edema of the face, arms, and chest

-

In severe cases: proptosis, glossal

and laryngeal edema and

obtundation.

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Superior vena cava

Superior vena cava

syndrome

syndrome

Chest radiographic
findings: widening of
the superior
mediastinum, most
commonly on the right
side.

However, a normal
chest radiographs is
still compatible with
the diagnosis if other
characteristic findings
are present

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Superior vena cava

Superior vena cava

syndrome

syndrome

Image with CT (MRI if can’t use contrast) –

computed tomography provides the most

reliable view of the mediastinal anatomy.

Tissue diagnosis (bronchoscopy,

percutaneous needle biopsy,

mediastinoscopy, and even thoracotomy)

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Superior vena cava

Superior vena cava

syndrome

syndrome

Treatment

Underlying cause:

-

Radiation therapy for SVCS caused by non – small cell lung

cancer and other metastatic solid tumors.

-

Chemotherapy for:

 Small cell carcinoma of the lung

 Lymphoma

-

Surgery – may provide immediate relief for patients in whom a

benign process is the cause.

-

Corticosteroids

- No benefit in patients with lung cancer
- May be useful at shrinking lymphoma masses

PROGNOSIS:

PROGNOSIS:

-

Clinical improvement - in most patients, although this

improvement may be due to the development of adequate

collateral circulation.

-

The mortality associated with SVCS does not relate to caval

obstruction, but rather to the underlying cause.

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Superior vena cava

Superior vena cava

syndrome

syndrome

Recurrent SVCS occurs in 10 to 30% of patients after initial therapy;

it may be palliated with the use of intravascular self-expanding

stents;

Early stenting may be necessery in patients with severe symptoms

Relative risk associated with this procedure: the prompt increase in

venous return after stenting may precipitate heart failure and

pulmonary oedema.

1.

1.

Venogram shows

Venogram shows

almost complete

almost complete

occlusion

occlusion

of the superior vena

of the superior vena

cava

cava

with dramatic

with dramatic

collateral

collateral

drainage through the

drainage through the

left

left

superior intercostal

superior intercostal

vein.

vein.

2.

2.

A stent mounted on

A stent mounted on

a

a

balloon was deployed

balloon was deployed

in

in

the superior vena cava.

the superior vena cava.

1.

1.

2.

2.

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Spinal cord compression

Spinal cord compression

Spinal cord compression develops in 1-5%

Spinal cord compression develops in 1-5%

(5-10%)

(5-10%)

of patients

of patients

with systemic cancer. It should be considered as

with systemic cancer. It should be considered as

emergency, as

emergency, as

treatment delays may result in irreversible

treatment delays may result in irreversible

paralysis and loss of bowel and bladder function

paralysis and loss of bowel and bladder function

.

.

Etiology:

Etiology:

-

Extradural metastases (95%) – ussually resuslts from tumor

Extradural metastases (95%) – ussually resuslts from tumor

involvement of the vertebral column. A tumor may

involvement of the vertebral column. A tumor may

occasionally metastasize to the epidural space without

occasionally metastasize to the epidural space without

bony involvement.

bony involvement.

-

Site of involvement: the segment most often involeved is

Site of involvement: the segment most often involeved is

the thoracic spine (70%), followed by the lumbosacral

the thoracic spine (70%), followed by the lumbosacral

(20%) and cervical spine (10%).

(20%) and cervical spine (10%).

-

Most common malignancies: lung, breast & prostate cancer,

Most common malignancies: lung, breast & prostate cancer,

multiple myeloma, lymphomas, melanoma, renal cancer

multiple myeloma, lymphomas, melanoma, renal cancer

and gastrourinary cancer.

and gastrourinary cancer.

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Spinal cord compression –

Spinal cord compression –

history

history

Early symptoms are often nonspecific and include local pain or

Early symptoms are often nonspecific and include local pain or

stiffness.

stiffness.

Gradually

Gradually

worsening back pain

worsening back pain

is the classic initial feature of spinal

is the classic initial feature of spinal

cord neoplastic disease in about 90% of adult patients who are

cord neoplastic disease in about 90% of adult patients who are

affected. Pain often precedes other symptoms associated with spinal

affected. Pain often precedes other symptoms associated with spinal

cord compression by days, weeks to 2-4 months. However, once

cord compression by days, weeks to 2-4 months. However, once

symptoms other than pain appear, symptom progression may be rapid.

symptoms other than pain appear, symptom progression may be rapid.

Pain is exacerbated by movement and by coughing or sneezing.

Pain is exacerbated by movement and by coughing or sneezing.

Radicular pain in the cervical or lumbosacral areas may be unilateral or

Radicular pain in the cervical or lumbosacral areas may be unilateral or

bilateral. Radicular pain from the thoracic roots is often bilateral and is

bilateral. Radicular pain from the thoracic roots is often bilateral and is

described by patients as a feeling of tight, band-like constriction

described by patients as a feeling of tight, band-like constriction

around the thorax and abdomen.

around the thorax and abdomen.

Typical cervical radicular pain radiates down the arm; in the lumbar

Typical cervical radicular pain radiates down the arm; in the lumbar

region the radiation is down the legs.

region the radiation is down the legs.

PATIENTS WITH CANCER WHO DEVELOP BACK

PATIENTS WITH CANCER WHO DEVELOP BACK

PAIN SHOULD BE EVALUATED FOR SPINAL

PAIN SHOULD BE EVALUATED FOR SPINAL

CORD

CORD

COMPRESSION AS QUICKLY AS POSSIBLE.

COMPRESSION AS QUICKLY AS POSSIBLE.

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Spinal cord compression –

Spinal cord compression –

physical

physical

The neurological signs accompanying cord compression vary

The neurological signs accompanying cord compression vary

according to:

according to:

-

the rapidity of development of compression

the rapidity of development of compression

-

the area of cord affected.

the area of cord affected.

Acute lesions often result in hypotonia and weakness.

Acute lesions often result in hypotonia and weakness.

Chronic lesions are more often associated with the classic upper

Chronic lesions are more often associated with the classic upper

motor neurone signs of hypertonia and hyper-reflexia.

motor neurone signs of hypertonia and hyper-reflexia.

The associated sensory loss is defined by the site of the lesion,

The associated sensory loss is defined by the site of the lesion,

but hyperaesthesia may be seen in the dermatome at the

but hyperaesthesia may be seen in the dermatome at the

level of the lesion.

level of the lesion.

More lateral lesions may result in a dissociative sensory loss—

More lateral lesions may result in a dissociative sensory loss—

that is, ipsilateral loss of joint position sense and

that is, ipsilateral loss of joint position sense and

proprioception with contralateral loss of pain and

proprioception with contralateral loss of pain and

temperature.

temperature.

Bladder and bowel disturbances often occur late, with the

Bladder and bowel disturbances often occur late, with the

exception of the cauda equina compression syndrome, in

exception of the cauda equina compression syndrome, in

which they are an early feature.

which they are an early feature.

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Spinal cord compression

Spinal cord compression

If cord compression is

If cord compression is

suspected the patient

suspected the patient

should be investigated with

should be investigated with

plain spinal radiography,

plain spinal radiography,

which may show evidence

which may show evidence

of lytic lesions (as, for

of lytic lesions (as, for

example, in myeloma).

example, in myeloma).

The definitive investigation

The definitive investigation

is magnetic resonance

is magnetic resonance

imaging to delineate the

imaging to delineate the

level of the lesion and to

level of the lesion and to

help plan further treatment

help plan further treatment

(MRI is to be done in first

(MRI is to be done in first

24 hours)

24 hours)

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Spinal Cord Compression

Clinical suspicion:

back pain

back pain

, weakness, motor/sensory

loss,

increased tone, hyperreflexia, loss of sphincter tone

Suspicious for
myelopathy

Symptomatic
treatment

Corticosteroids

10

10

-

-

100 mg bolus

100 mg bolus

,

then 4 mg
qid
Assess pain control
Need for foley
catheter
Review recent bone
scan if available
Radiation oncology
consult

MRI scan

MRI scan

Spinal cord

Spinal cord

compressio

compressio

n present

n present

on MRI

on MRI

scan?

scan?

Urgent radiation oncology
evaluation
Consider neurosurgical
consult for:
- Previously radiated area
- No previous history of
cancer
(for tissue diagnosis)
- Spinal instability or bony
compression of spinal cord
- Radioresistant tumors
(renal, melanoma)
- Neurologic deterioration on
radiation

Yes

No

Yes

No

Reassess patient
Consider other
pathology for spinal
cord dysfunction

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Spinal Cord Compression

Radiation therapy plus

Radiation therapy plus

glucocorticoids is generally the initial

glucocorticoids is generally the initial

treatment of choice.

treatment of choice.

Up to 75% of patients treated when

Up to 75% of patients treated when

still ambulatory remain ambulatory,

still ambulatory remain ambulatory,

but only 10% of patients with

but only 10% of patients with

paraplegia recover walking capacity.

paraplegia recover walking capacity.

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Hyperviscosity Syndrome

Hyperviscosity Syndrome

Refers to the clinical sequelae of increased blood

Refers to the clinical sequelae of increased blood

viscosity. Increased serum viscosity usually results

viscosity. Increased serum viscosity usually results

from increased circulating serum immunoglobulins and

from increased circulating serum immunoglobulins and

can be seen in Waldenström macroglobulinemia and

can be seen in Waldenström macroglobulinemia and

multiple myeloma.

multiple myeloma.

Is associated most commonly with plasma cell

Is associated most commonly with plasma cell

dyscrasias and is due to the large size of the excess

dyscrasias and is due to the large size of the excess

immunoglobulin M (IgM) paraproteins in these

immunoglobulin M (IgM) paraproteins in these

disorders. Waldenström macroglobulinemia is the most

disorders. Waldenström macroglobulinemia is the most

common cause. Rarely, the disease can occur in

common cause. Rarely, the disease can occur in

multiple myeloma (especially with myeloma proteins

multiple myeloma (especially with myeloma proteins

of the IgA and IgG types) or connective tissue

of the IgA and IgG types) or connective tissue

diseases.

diseases.

Clinical presentation of the syndrome:

Clinical presentation of the syndrome:

-

mucous membrane bleeding,

mucous membrane bleeding,

-

retinopathy,

retinopathy,

-

neurologic symptoms.

neurologic symptoms.

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Hyperviscosity Syndrome

Hyperviscosity Syndrome

History:

History:

Tendency to bleed is the most common symptom of

Tendency to bleed is the most common symptom of

hyperviscosity syndrome.

hyperviscosity syndrome.

Spontaneous gum bleeding

Spontaneous gum bleeding

Epistaxis

Epistaxis

Rectal bleeding

Rectal bleeding

Menorrhagia

Menorrhagia

Persistent bleeding after minor procedures

Persistent bleeding after minor procedures

Visual changes range from blurred vision to vision loss.

Visual changes range from blurred vision to vision loss.

Neurologic manifestations are frequent and varied. The

Neurologic manifestations are frequent and varied. The

neurologic symptoms of hyperviscosity have been

neurologic symptoms of hyperviscosity have been

referred to as the Bing-Neal syndrome.

referred to as the Bing-Neal syndrome.

Vertigo

Vertigo

Hearing loss

Hearing loss

Paresthesias

Paresthesias

Ataxia

Ataxia

Headaches

Headaches

Seizures

Seizures

Somnolence progressing to stupor and coma

Somnolence progressing to stupor and coma

Other manifestations may include heart failure,

Other manifestations may include heart failure,

fatigue, and anorexia.

fatigue, and anorexia.

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Hyperviscosity Syndrome

Hyperviscosity Syndrome

Physical:

Physical:

Physical findings are

Physical findings are

related to the 3 major organ systems

related to the 3 major organ systems

involved.

involved.

Bruises, epistaxis, or gum bleeding

Bruises, epistaxis, or gum bleeding

may be noted.

may be noted.

Ophthalmic examination may reveal

Ophthalmic examination may reveal

decreased visual acuity, dilated

decreased visual acuity, dilated

retinal veins, sausage-linked retinal

retinal veins, sausage-linked retinal

veins, or retinal hemorrhages.

veins, or retinal hemorrhages.

Neurological examination may reveal

Neurological examination may reveal

various findings, including

various findings, including

diminished mental status, confusion,

diminished mental status, confusion,

ataxia, or nystagmus.

ataxia, or nystagmus.

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Hyperviscosity Syndrome

Hyperviscosity Syndrome

Lab Studies:

Lab Studies:

Serum viscosity.

Serum viscosity.

Serum viscosity is

Serum viscosity is

diagnostic in evaluating

diagnostic in evaluating

hyperviscosity syndrome.

hyperviscosity syndrome.

The reference range is 1.4-

The reference range is 1.4-

1.8 units.

1.8 units.

Symptoms usually are not

Symptoms usually are not

seen before the viscosity

seen before the viscosity

reaches 4 units, and

reaches 4 units, and

hyperviscosity syndrome

hyperviscosity syndrome

usually presents with a

usually presents with a

serum viscosity greater

serum viscosity greater

than 5 units.

than 5 units.

P

P

eripheral blood smear.

eripheral blood smear.

Rouleaux formation

Rouleaux formation

is

is

often present with

often present with

increased serum viscosity.

increased serum viscosity.

Total protein level;

Total protein level;

IgG, IgA,

IgG, IgA,

IgM

IgM

serum

serum

levels.

levels.

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Hyperviscosity Syndrome

Hyperviscosity Syndrome

Plasmapheresis is the treatment of

Plasmapheresis is the treatment of

choice for hyperviscosity syndrome.

choice for hyperviscosity syndrome.

As plasmapheresis removes the

As plasmapheresis removes the

circulating paraproteins, the serum

circulating paraproteins, the serum

viscosity decreases and symptoms

viscosity decreases and symptoms

improve.

improve.

While arranging for plasmapheresis,

While arranging for plasmapheresis,

treat hemorrhage, CHF, and metabolic

treat hemorrhage, CHF, and metabolic

imbalances with standard therapies.

imbalances with standard therapies.

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Hyperviscosity Syndrome

Hyperviscosity Syndrome

Blood viscosity is a function of the

Blood viscosity is a function of the

concentration and composition of its

concentration and composition of its

components. A marked increase in plasma

components. A marked increase in plasma

proteins (for example, monoclonal

proteins (for example, monoclonal

immunoglobulin in myeloma) or cellular

immunoglobulin in myeloma) or cellular

constituents (for example, white blood cells

constituents (for example, white blood cells

in acute leukemia) will raise the overall

in acute leukemia) will raise the overall

blood viscosity.

blood viscosity.

Blood viscosity is increased also in:

Blood viscosity is increased also in:

- Polycythaemia

- Polycythaemia

- High white cell count (hyperleucocytosis)

- High white cell count (hyperleucocytosis)

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Hyperleukocytosis

Hyperleukocytosis

Definition: peripheral leukocyte count greater

Definition: peripheral leukocyte count greater

than 100,000/mm3 (100 G/L).

than 100,000/mm3 (100 G/L).

Hyperleukocytosis is present at diagnosis in 5-

Hyperleukocytosis is present at diagnosis in 5-

13% of patients with AML, 10-30% of patients

13% of patients with AML, 10-30% of patients

with ALL, and nearly all adults with

with ALL, and nearly all adults with

CML

CML

.

.

Respiratory complications are a more

Respiratory complications are a more

prominent feature of elevated leukocyte counts

prominent feature of elevated leukocyte counts

in patients with AML.

in patients with AML.

Hemorrhagic complications and death rates

Hemorrhagic complications and death rates

significantly increase when peripheral

significantly increase when peripheral

leukocyte counts are greater than

leukocyte counts are greater than

100,000/mm3 in the context of AML and

100,000/mm3 in the context of AML and

greater than 300,000-400,000/mm3 in the

greater than 300,000-400,000/mm3 in the

context of ALL.

context of ALL.

Leucocytostasis c

Leucocytostasis can also occur in CLL when WBC >

400,000/mm3.

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Hyperleukocytosis

Hyperleukocytosis

Physical findings result

Physical findings result

from the increased

from the increased

viscosity associated with

viscosity associated with

blast cell aggregates and

blast cell aggregates and

thrombi in combination

thrombi in combination

with damage to vessels

with damage to vessels

and secondary

and secondary

hemorrhage. Resultant

hemorrhage. Resultant

clinical findings primarily

clinical findings primarily

include respiratory and

include respiratory and

neurologic signs.

neurologic signs.

Respiratory signs:

Respiratory signs:

-

dyspnea

dyspnea

-

hypoxia.

hypoxia.

Neurologic signs:

Neurologic signs:

-

focal deficit,

focal deficit,

-

ataxia,

ataxia,

-

agitation,

agitation,

-

confusion,

confusion,

-

delirium,

delirium,

-

stupor.

stupor.

Other signs include:

Other signs include:

-

plethora,

plethora,

-

cyanosis,

cyanosis,

-

papilledema, and retinal

papilledema, and retinal

artery or retinal vein

artery or retinal vein

distension.

distension.

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Hyperleukocytosis

Hyperleukocytosis

Specific antileukemic therapy is the treatment of choice

Specific antileukemic therapy is the treatment of choice

for decreasing the peripheral leukocyte count.

for decreasing the peripheral leukocyte count.

In the absence of definitive antileukemic therapy,

In the absence of definitive antileukemic therapy,

leukophoresis may be considered;

leukophoresis may be considered;

The goal of the therapies is to decrease blood viscosity

The goal of the therapies is to decrease blood viscosity

and the metabolic risks associated with a large tumor

and the metabolic risks associated with a large tumor

burden.

burden.

L

L

eukophoresis may be considered if a delay is expected

eukophoresis may be considered if a delay is expected

in initiating specific antileukemic therapy and leukocyte

in initiating specific antileukemic therapy and leukocyte

counts are greater than 100,000/mm3 in patients with

counts are greater than 100,000/mm3 in patients with

A

A

M

M

L or 300,000-400,000/mm3 in patients with ALL.

L or 300,000-400,000/mm3 in patients with ALL.

PRBC transfusions increase the viscosity of blood

PRBC transfusions increase the viscosity of blood

and should be avoided, if possible, in the context

and should be avoided, if possible, in the context

of hyperleukocytosis.

of hyperleukocytosis.

Platelet transfusions do not significantly change

Platelet transfusions do not significantly change

the viscosity of circulating blood, and platelets

the viscosity of circulating blood, and platelets

may be transfused safely if indicated.

may be transfused safely if indicated.

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Leukoreduction Apheresis

Leukoreduction Apheresis

(LRA)

(LRA)

The process of removing

The process of removing

unwanted WBC or blasts

unwanted WBC or blasts

from the circulation

from the circulation

The procedure is indicated

The procedure is indicated

for the rapid correction of

for the rapid correction of

hyperleukocytosis

hyperleukocytosis

.

.

One procedure generally

One procedure generally

removes between 20-80%

removes between 20-80%

of WBC by processing 7-10

of WBC by processing 7-10

liters of blood

liters of blood

Indications for LRA

Indications for LRA

:

:

-

-

Hyperleukocytosis

Hyperleukocytosis

WBC/Blast layer

WBC/Blast layer

Blast cell


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