No abstract

\s=b\An 11-year-old black boy with sickle cell anemia developed profound pancytopenia during the course of his disease, but fully recovered therefrom. The patient was receiving anticonvulsant drugs for a seizure disorder secondary to a "stroke," and, therefore, a drug-related marrow aplasia cannot be ruled out.(Am J Dis Child 129: [1195][1196] 1975) The aplastic crisis in sickle cell anemia18 usually represents a temporary failure of erythrocyte production. Aplastic crisis is a mis¬ nomer, since there is only an acute erythroblastopenia2 3 and reticulocytopenia.7 Leukopenia is quite uncom¬ mon, and thrombocytopenia is ex¬ tremely rare. The present report concerns a patient with homozygous sickle cell anemia who had profound pancytopenia during the course of his disease, but fully recovered there¬ from. The patient was receiving anti¬ convulsant drugs, and, therefore, a drug-related marrow aplasia cannot be ruled out.An 11-year-old black boy, known to have sickle cell anemia since the age of 8 months, was hospitalized with high fever, generalized malaise, and lethargy.At 2V4 years of age, the patient suffered a cerebral thrombosis that resulted in partial aphasia, right hemiparesis, and a seizure disorder for which he was treated with anticonvulsants, most recently primidone (Mysoline) (250 mg daily), mephenytoin (Mesantoin) (200 mg daily), and phénobarbital (128 mg daily). The patient was acutely ill, febrile, and tachypneic. Mucous membranes were pale and sclerae were slightly jaundiced. Gingivae were severely hypertrophie. There was generalized lymphadenopathy. The heart was enlarged and a loud systolic ejection murmur was heard all over the precordium. The lungs were clear to per¬ cussion and auscultation. The liver was palpable 12 cm below the right costal mar¬ gin. The spleen was not felt. There was also hemiparesis and contracture de¬ formity of the right upper and lower ex¬ tremities, with increased deep tendon reflexes.The hemoglobin level was 5.5 gm/100 ml; hematocrit value, 16%; red blood cell count, 1.6 million/cu mm; and white blood cell count, 5,600/cu mm with 53% neutrophils, 43% lymphocytes (of which half were atyp¬ ical), 2% eosinophils, and 2% plasmacytes. The reticulocyte count was 1.5%, and plate¬ let count was 208,000/cu mm. Target cells, sickle cells, and a few burr cells were seen on the peripheral blood smear. Serial blood counts are shown in the Figure. Bone marrow aspirate was extremely hypoplastic. Cultures from blood, nasopharynx, urine, and stool were sterile.Results of CSF examination were within normal limits, and culture was sterile. An¬ ticonvulsant therapy was discontinued and ampicillin sodium, 1 gm daily, and folie acid, 5 mg daily, were administered. On the third day of hospitalization, the leuko¬ cyte count was 3,700/cu mm and the plate¬ let count was 137,000/cu mm.On the 18th day, the platelet nadir of 20,000/cu mm and leukocyte nadir of 1,800/cu mm were reached. Viral, fungal, and protozoal studies in blood, urine, and stool were also negative. The pancytopenia slowly rev...