Case 478 -- A 7 year old boy with massive splenomegaly

Contributed by Siobhan O’Connor, MD, Lydia Contis, MD, K Michael Gibson, PhD, David Finegold, MD and Mohamed Virji, MD, PhD
Published on line in September 2006


The patient is a 7 year old boy of Turkish descent who was diagnosed in Russia with splenomegaly and anemia at the age of 8 months. He began having seizures, usually associated with fever, at the age of 6 months. These continued every two to four months until the age of five. He has not had any seizures for two years, and his medications have been discontinued. He was hospitalized at an outside hospital in the fall of 2005 due to severe anemia.

Physical examination by a pediatric hematologist/oncologist in late 2005 demonstrated massive splenomegaly to 15 cm below the left costal margin. The liver was palpable to approximately 1 cm below the right costal margin. Abdominal ultrasound confirmed marked splenomegaly, and the liver was enlarged at its inferior portion.

CBC from late 2005:

Iron Studies:

Liver function tests were within normal limits, and viral hepatitis studies were negative. As noted above, the CBC indicated pancytopenia. The patient was started on oral iron for suspected iron deficiency anemia.

Magnetic resonance imaging performed April 2006 confirmed the patient's massive splenomegaly. Several levels of the MRI are shown here to better illustrate the size of the spleen. No hepatomegaly was noted initially, but was subsequently appreciated.


A bone marrow biopsy was performed 1/31/2006 to due to the patient's persistent pancytopenia and splenomegaly. The specimen was sent to pathology accompanied by peripheral blood.

Peripheral Blood (Complete Blood Cell Count/Differential):

Bone Marrow Differential on aspirate smear:



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