Post-transfusion graft versus host disease in an infant with severe combined immunodeficiency

Graft Versus Host Disease [GVHD] occurs when immunologically competent lymphocytes are introduced into an immunoincompetent host. GVHD occurs after allogenic Bone Marrow Transfusion [BMT] and less often after transfusion of nonirradiated cellular blood components. This 6 month-old male baby was referred with persistent pneumonia and poor weight gain since 4 months of his life. The parents are in close consanguinity and experience the death of their first baby who was a girl and expired with infection at early infancy. The patient's data showed severe lymphocytopenia, severe hypogammaglobulinemia [undetectable serum IgG and IgM] and low CD4 count by flow cytometry. These findings are consistent with Severe Combined Immuno Deficiency [SCID], which is a severe and fatal T-cell immunodeficiency. Intravenous; immunoglobulin, antibiotics and systemic antifungal therapy were administered. Unfortunately he had received an emergency blood transfusion before confirmation of diagnosis. Two weeks later he developed pancytopenia, generalized erythematous scaly skin rash, hepatomegaly and cholestatic jaundice. Cyclosporine and steroid started due to suspected post-transfusion Graft Versus Host Disease [GVHD], but there was not enough time to observed the effect of drugs and he was expired. There is an increased danger from TA-GVHD in part because of the frequent failure of physicians to recognize, and producing recipient BM aplasia. Post-transfusion,GVHD is fatal in more than 90% of cases, primarily because of producing BM aplasia. The use of irradiated blood [>2500 cGy] is now recommended in high risk group including BMT recipients, congenital immunodeficiency syndromes, intrauterine transfusion, transfusion from first-degree blood relatives, and in Hodgkin's lymphoma. GVHD continues to be a rare but extremely serious complication of blood transfusion

Anterior hypopituitarism associated with sickle cell trait in a 13-year-old boy

We are reporting a 13-year-old boy with hypopituitarism associated with sickle cell trait. The patient was referred with growth arrest since 10-year of age, and had evidence of growth hormone deficiency, hypoadrenalism and secondary hypothyroidism. A magnetic resonance imaging study revealed an empty sella with no evidence of tumor. Review of the literature showed three patients with sickle cell trait and hypopituitarism, all being in the third decade or later of life. To our knowledge, this patient is the first case of sickle cell trait with anterior hypopituitarism in childhood. This case supports a causal relationship between sickle cell trait and hypopituitarism