Pearson syndrome associated with hemophagocytic syndrome in a child.

A seven-month-old boy was admitted to a local health center with a two-week history of fever, cough, vomiting, and diarrhea. The patient was the first child of healthy, unrelated parents. Pancytopenia, increased activated partial thromboplastin time (aPTT) and international normalized ratio (INR), and acidosis were detected during local health care evaluation, and the patient was referred to our hospital with the diagnosis of metabolic disorder. Physical examination revealed toxic appearance, lethargy, paleness, and hepatomegaly palpable 4 cm below the right costal margin. The results of the laboratory examinations were as follows: Hematological studies showed hemoglobin 7.4 g/dl, platelet 93x109/L, white blood cells 7.4x109/L with a differential count of 21% neutrophils, 70% lymphocytes, 9% monocytes, mean corpuscular volume (MCV) 91.3 fl, and red cell distribution width (RDW) 14.7. Peripheral blood smears showed burr cell, schistocyte, and acanthocyte. Serum alanine aminotransferase was 237 U/L (5-40 U/L), aspartate aminotransferase 506 U/L (8-33 U/L), total bilirubin 1.46 mg/dl (0.10-1.20 mg/dl), conjugated bilirubin 1.04 mg/dl (0-0.30 mg/dl), blood urea nitrogen 2.0 mg/dl (5-18 mg/dl), creatinine 0.28 mg/dl (0.6-1.2 mg/dl), sodium 127 mEq/L (138-145 mEq/L), potassium 3.21 mEq/L (3.4-4.7 mEq/L), chloride 95 mEq/L (95-110 mEq/L), calcium 13.2 mg/dl (8.6-10.2 mg/dl), phosphorus 2.0 mg/dl (2.3-4.7 mg/dl), blood pH 7.282 (7.35-7.45), bicarbonate 7 mmol/L (21-28 mmol/L), lactic acid 69.4 mg/dl (10-14 mg/dl), and pyruvic acid 2.18 mg/dl (0.51.0 mg/dl). Bone marrow aspiration smear showed vacuolization of hematopoietic preReceived: 13.09.2007 • Accepted: 04.10.2007 ©Turkish Society of Hematology IMAGE IN HEMATOLOGY