April 5, 2013

Hematology Emergency: Hepatospleenomegaly with Hyperleukocytosis

35 years old lady, Para 2, on contraceptive, presented with left abdominal mass and discomfort which only being noticed since 5 days ago. Apart from that she denied any history of fever or recurrent fever, no constitutional symptoms, no sign and symptoms of connective tissue disease, no family history of malignancy and no altered sensorium or loss of consciousness.

Examination reveals a moderate built lady with fully conscious and oriented, pallor but stable vital sign, massive spleenomegaly which is firm in nature, liver palpable 4 finger breadth with firm in consistency, regular margin. The rest of abdomen system, cardiovascular examination and respiratory examination reveal no significant finding.

Bedside ultrasounds confirm the finding of Hepatospleenomegaly in which both the organ shows normal parenchyma and no focal lesion.

The full blood count shows the finding of 1) Normochromic, normocytic anemia 2) Hyperleukocytosis with predominant neutrophils. Blood smear for malaria parasite is negative and LDH was 1600.

Hyperleukocytosis refers to a laboratory abnormality that has been variably defined as a total leukemia blood cell count greater than 50 x 10^9/L (50,000/microL) or 100 x 10^9/L (100,000/microL). In contrast, leukostasis (also called symptomatic hyperleukocytosis) is a medical emergency most commonly seen in patients with acute myeloid leukemia (AML) or chronic myeloid leukemia (CML) in blast crisis. It is characterized by an extremely elevated blast cell count and symptoms of decreased tissue perfusion. (Charles A Schiffer, UpToDate)

Leukostasis is a pathologic diagnosis in which white cell plugs are seen in the microvasculature. Clinically, leukostasis is typically diagnosed empirically when a patient with leukemia and hyperleukocytosis presents with respiratory or neurological distress. Prompt treatment is indicated since, if left untreated, the one-week mortality rate is approximately 20 to 40 percent. (Charles A Schiffer, UpToDate)

Leukostasis occurs when the high white cell counts and leukocyte aggregates and lead to increase blood viscosity which later resulting in stasis of blood in smaller blood vessel. This will then lead to neurological, pulmonary and gastrointestinal complication. It also increase risk for tumor lysis syndrome.

Hyperleukocytosis is more common in acute leukemia compared to chronic leukemia. It presents in 10-20 percent acute myeloid leukemia patient, 10-30% of patient with acute lymphoblastic leukemia.

Initial management includes 1) aggressive hydration, 2) Prevention of tumor lysis syndrome by a) Allopurinol, b) Recombinant Urate Oxidase (rasburicase), c) Alkalization of urine, 3) correction of metabolic abnormalities, 4) correction of DIVC or thrombocytopenia if present.

Leukapheresis is the treatment of choice for a very high count, or in patients with symptomatic hyperleukocytosis. Hydroxyurea is an anti metabolites drug should be used judiciously as it also may trigger acute tumor lysis syndrome. Packed cell transfusion should only be reserved in symptomatic anemia or hemoglobin less than 7 as it may worsened the hyper viscosity of blood.


1) Charles A Schifer, Hyperleukocytosis and leukostasis", UpToDate accessed on 5/4/13, 9.00 PM

2) Jain R, Bansal D, Marwaha RK., "Hyperleukocytosis: emergency management", Indian J Pediatr. 2013 Feb

3) Michael J. Mauro, "Hyperleukocytosis in Acute Myeloid Leukemia",  N Engl J Med 349;8, 2003

4) H Singh et al, "Hyperleukocytosis Associated Pulmonary Leukostasis in Acute Leukaemia", JAPI, vol 54, 2006

5) Navneet S Majhail et al, "cute leukemia with a very high leukocyte count: Confronting a medical emergency", CLEVELAND CLINIC JOURNAL OF MEDICINE 2004

1 comment:

Ya Allah! Permudahkanlah aku untuk menuntut ilmuMu, memahaminya, mengingati dan menyebarkannya. Berkatilah ilmu itu dan tambahkanlah ia. Amin.