July 1996

Pathological Case of the Month

Author Affiliations

From the Department of Pathology, Division of Hematopathology (Drs Quintana, Contis, and Penchansky), and Department of Pediatrics, Division of Hematology/Oncology (Dr Koehler), Childrens Hospital of Pittsburgh University of Pittsburgh School of Medicine, Pittsburgh, Pa.

Arch Pediatr Adolesc Med. 1996;150(7):763-764. doi:10.1001/archpedi.1996.02170320109020

A 14-YEAR-OLD black girl with monozygous sickle cell anemia was admitted to Children's Hospital of Pittsburgh, Pa, with abdominal and bone pain. Results of physical examination were normal. Within a 3-month period, she had four painful crises, which required hospitalization; they were associated with a high platelet count, 2000×109/L. Her hemoglobin level ranged from 7.2 to 7.8 g/L and white blood cell count from 15 to 22×109/L. The peripheral blood smear showed anisopoikilocytosis, including abundant sickle cells, a leukoerythroblastic picture, and marked thrombocytosis with giant platelets and circulating sickle cells (Figure 1). Howell-Jolly bodies were also seen. Because of a persistently high platelet count, above 1000×109/L, an iliaccrest bone marrow aspiration and bone marrow biopsy were performed to rule out a myelodysplastic syndrome. The bone marrow aspirate smear was hypercellular, with a decreased myeloid-erythroid ratio of 0.9. There was megaloblastoid erythroid maturation with moderate

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