Emergencies in

hematology

M. Zawartko, M.D.

Department of Hematology,

Pomeranian Medical University,

Szczecin

Emergencies in hematology



ATLS



Hypercalcemia



Spinal cord compression



Superior vena cava syndrome



Leukocytostasis



Hyperviscosity



Pericardial effusion/tamponade



Intestinal obstruction



Urinary obstruction



Malignant biliary obstruction



Increased intracranial pressure



Neoplastic menigitis



Other….

ATLS – Acute tumor lysis

syndrome



Is characterized by various combinations of:

hyperuricemia;

Hypergl

cemia;

Hyperkalemia;

Hyperphosphatemia;

Hypocalcemia;

Lactic acidosis.



Is caused by the destruction of a large

number of rapidly proliferating neoplastic

cells.



Frequently: acute renal failure develops as a

result of the syndrome.

-Uric acid
-Glucose
-Potassium
-Phosphate

s
-Lactic acid

ATLS – Acute tumor lysis

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)

after chemotherapy;



In rare cases – spontaneous necrosis of

malignancies causes ATLS – prior to chemotherapy.

ATLS – Acute tumor lysis

syndrome

Hyperuricemia:



May be present at the time of chemotherapy



Effective treatment kills malignant cells and

leads to increased serum uric acid levels

from the turnover of nucleic acids.



Uric acid can precipitate in the tubules,

medulla and collecting ducts of the kidney

leading to renal failure.



Lactic acidosis and dehydratation may

contribute to the precipitation of uric acid in

the renal tubules.

ATLS – Acute tumor lysis

syndrome

Hyperphosphatemia



Produces reciprocal depression in serum calcium



Hypocalcemia – symptoms: neuromuscular

irritability, tetany;



Deposition of calcium phosphate in the kidney

and hyperphosphatemia may cause renal

failure.

Hyperkalemia

In patients with renal failure may rapidly be life

– threatening.

Can cause ventricular arrhythmias and sudden

death.

ATLS – Acute tumor lysis

syndrome



The most important predictive factors:

tumor burden: hyperuricemia and high

levels of lactate dehydrogenase

(LDH>1500 U/l);

Renal function



Recognition & prevention are the most

important steps in the management of

this syndrome.

ATLS – Acute tumor lysis

syndrome

Prophylaxis



Maintain hydratation by administration of ivf

at 3000 ml/m2 per day



Keep urine pH at 7,0 or greater by

administration of sodium bicarbonate



Administer allopurinol at 300 mg/m2 per day



Monitor serum chemistry.

Start chemotherapy when serum uric acid <8,0

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

>7,0.

If an urgent anticancer therapy is needed

consider dialysis.

ATLS – Acute tumor lysis

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

Xanthin
e
oxidase

Uric Acid

Allantoin

Urate Oxidase

Allopurin
ol

Hypoxanthi

Xanthine
oxidase

Rasburicase

ATLS – Acute tumor lysis

syndrome



Despite aggressive

prophylaxis ATLS

and/or oliguric or

anuric renal failure

may occur;



Dialysis is often

necessary and

should be considered

early in the course.



The prognosis is

good, and renal

function recovers

after the uric acid

level is lowered to <

10 mg/dl.

If:



Serum K+ > 6mEq/l



Serum uric acid >10

mg/dl



Serum

creatinine>10 mg/dl



Serum

phosphate>10mg/dl

or increasing

symptomatic

hypocalcemia

present



Treatment

Begin

hemodialysis

Superior vena cava syndrome

(SVCS)



The superior vena

cava syndrome is

the group of

symptoms that

result from

compression of the

large vein (superior

vena cava) that

transmits blood to

the heart from the

head, neck and

upper extremities.

Superior vena cava

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!!

- Others (primary mediastinal

germ cell, thymoma)



Infection



Thrombosis

Superior vena cava

syndrome



Up to 60% with SVCS

will not have prior

cancer diagnosis



History:

Dyspnea

facial swelling, head fullness,

cough, arm swelling, chest pain,

dysphagia,

orthopnea,

distorted vision,

hoarseness,

stridor,

headache,

nasal stuffiness,

nausea,

light-headedness.



Physical:

venous distension of the neck

and chest wall,

facial edema,

upper extremity edema,

mental changes,

plethora,

cyanosis,

papilledema,

stupor,

oma

ending forward or lying down

may aggravate the symptoms

and signs.

Superior vena cava

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

Superior vena cava

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)

Superior vena cava

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:

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.

Spinal cord compression

evelops in 1-5% (5-10%) of patients with systemic cancer.

It should be considered as emergency, as

treatment delays

may result in irreversible paralysis and loss of bowel and

bladder function

Etiology:

Extradural metastases (95%) – usually tumor

involvement of the vertebral column. A tumor may

occasionally metastasize to the epidural space without

bony involvement.

Site of involvement:

thoracic spine (70%)

lumbosacral spine (20%)

cervical spine (10%)

Most common malignancies: lung, breast & prostate

cancer, multiple myeloma, lymphomas, melanoma, renal

cancer and gastrourinary cancer.

Spinal cord compression –

history



Worsening back pain

- in about 90% of adult patients.



Pain often precedes other symptoms associated with spinal cord

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

symptoms other than pain appear, symptom progression may be

rapid.



Pain is exacerbated by movement and by coughing or sneezing.



Radicular pain in the cervical or lumbosacral areas may be

unilateral or bilateral.



Radicular pain from the thoracic roots is often bilateral and is

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

around the thorax and abdomen.



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

region the radiation is down the legs.



Bladder and bowel disturbances often occur late, with the exception

of the cauda equina compression syndrome, in which they are an

early feature.

PATIENTS WITH CANCER WHO DEVELOP BACK

PAIN SHOULD BE EVALUATED FOR SPINAL

CORD

COMPRESSION AS QUICKLY AS POSSIBLE.

Spinal cord compression



If cord compression is

suspected the patient

should be investigated with

plain spinal radiography,

which may show evidence

of lytic lesions (as, for

example, in myeloma).



The definitive investigation

is magnetic resonance

imaging to delineate the

level of the lesion and to

help plan further treatment

(MRI is to be done in first

24 hours)

Spinal Cord Compression

Clinical suspicion:

back pain

, weakness, motor/sensory

loss,

increased tone, hyperreflexia, loss of sphincter tone

Suspicious for
myelopathy

Symptomatic
treatment

Corticosteroids

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

Spinal cord

compressio

n present

on MRI

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

Reassess patient
Consider other
pathology for spinal
cord dysfunction

Spinal Cord Compression



Radiation therapy plus

glucocorticoids is generally the initial

treatment of choice.



Up to 75% of patients treated when

still ambulatory remain ambulatory,

but only 10% of patients with

paraplegia recover walking capacity.

Hyperviscosity Syndrome



Blood viscosity is a function of the

concentration and composition of its

components. A marked increase in plasma

proteins or cellular constituents (for

example, white blood cells in acute

leukemia) will raise the overall blood

viscosity.



Increased serum viscosity

due to

increased

circulating serum immunoglobulins can be

seen in Waldenström macroglobulinemia

(IgM) and multiple myeloma.



Blood viscosity is increased also in:

- Pol

cythaemia

- High white cell count (hyperleucocytosis)

Hyperviscosity Syndrome

History:



Tendency to bleed is the most common

symptom of hyperviscosity syndrome.

►

Spontaneous gum bleeding

►

Epistaxis

►

Rectal

bleeding

►

Menorrhagia

►

Persistent bleeding

after minor procedures



Visual changes range from blurred vision to

vision loss.



Neurologic manifestations are frequent and

varied. The neurologic symptoms of

hyperviscosity have been referred to as the

Bing-Neal syndrome.



Other manifestations may include heart

failure, fatigue, and anorexia.

Hyperviscosity Syndrome



Physical:



Bruises, epistaxis, or gum bleeding.



Ophthalmic examination may reveal

decreased visual acuity, dilated

retinal veins, sausage-linked retinal

veins, or retinal hemorrhages.



Neurological examination may reveal

various findings, including

diminished mental status, confusion,

ataxia, or nystagmus

, h

eadaches

eizures

, s

omnolence progressing to

stupor and coma.

Hyperviscosity Syndrome

Lab Studies:



Total protein level;

IgG, IgA,

IgM

serum

levels.



eripheral blood

smear.

Rouleaux

formation

is often

present with

increased serum

viscosity.

Hyperviscosity Syndrome



Plasmapheresis is the treatment of

choice for paraproteins related

hyperviscosity syndrome.

–

As plasmapheresis removes the circulating

paraproteins, the serum viscosity

decreases and symptoms improve.

–

While arranging for plasmapheresis, treat

hemorrhage, CHF, and metabolic

imbalances with standard therapies.

Hyperleukocytosis



Definition: peripheral leukocyte count greater

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



Hyperleukocytosis is present at diagnosis in 5-

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

with ALL, and nearly all adults with

CML



Leucocytostasis c

Leucocytostasis can also occur in CLL when

WBC > 400,000/mm3.



Physical findings result from the increased

viscosity associated with blast cell aggregates

and thrombi in combination with damage to

vessels and secondary hemorrhage. Resultant

clinical findings primarily include respiratory

and neurologic signs.

Hyperleukocytosis



Respiratory signs:

dyspnea

hypoxia.



Neurologic signs:

focal deficit,

ataxia,

agitation,

confusion,

delirium,

stupor.

Other signs include:

plethora,

cyanosis,

papilledema, and

retinal artery or

retinal vein

distension.

Hyperleukocytosis



Specific antileukemic therapy is the

treatment of choice for decreasing the

peripheral leukocyte count.



PRBC transfusions increase the

viscosity of blood and should be

avoided, if possible, in the context

of hyperleukocytosis.

Platelet transfusions do not

significantly change the viscosity of

circulating blood, and platelets may

be transfused safely if indicated.

Leukoreduction Apheresis

(LRA)



The process of

removing unwanted

WBC or blasts from the

circulation



The procedure is

indicated for the rapid

correction of

hyperleukocytosis



One procedure

generally removes

between 20-80% of

WBC by processing 7-

10 liters of blood

WBC/Blast layer

Blast cell

Hypercalcemia

It occurs in an estimated 10% to 20% of all

adults with cancer.



Causes

increase in the amount of calcium absorbed

from the bones,

and an inability of the kidneys to excrete

excess calcium.

ome cancer cells secrete substances that

cause calcium to be absorbed into the

bloodstream from bones.

Immobility, dehydration, anorexia, nausea,

and vomiting may also increase calcium levels.

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

Hypercalcemia of Malignancy

Suspect hypercalcemia

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

Hypercalcemia of Malignancy

Treatment

(treat underlying malignancy when

possible)

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

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

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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