Summary:Until recently, therapy for patients with severe congenital dyserythropoietic anemia (CDA) has been limited to blood transfusions and chelation therapy. Three children with transfusion-dependent CDA type I underwent allogeneic stem cell transplantation (SCT) from matched sibling donors. Conditioning was with cyclophosphamide 50 mg/kg/day for 4 days, busulphan 4 mg/kg/day for 4 days, and antithymocyte globulin (ATG) 30 mg/kg for four doses pre-SCT. All patients engrafted and are alive, and transfusion independent. To our knowledge, this is the first report of successful SCT in the management of CDA type I.

Summary.Several recent studies have demonstrated Kaposi's sarcoma-associated herpes virus (KSHV), also known as putative human herpes virus-8 (HHV-8), DNA in various epidemiologic forms of Kaposi's sarcoma (KS), including AIDS-associated, classic, and endemic types. Risk of developing KS in non-HIV-infected immunosuppressed hosts, such as patients following solid organ transplantation, is also significantly higher compared to normal individuals. We have retrospectively evaluated 28 organ transplant patients with KS (23 cutaneous and five visceral) for the presence of KSHV genome by polymerase chain reaction (PCR) amplification of DNA isolated from formalin-fixed, paraffinembedded archival tissue samples. 27/28 KS patients were positive for the presence of KSHV. In four KS patients, tissue samples with no histologic evidence of KS were also analysed for KSHV. No evidence of positivity in three samples was noted, but one patient had weak positive amplification products on DNA samples isolated from a gastric biopsy with chronic gastritis and lymph node with sinus histiocytosis. These data support the association of KSHV with KS developing in non-HIV-infected immunosuppressed patients, similar to other forms of KS, and suggest that KSHV may play a significant role in the development of all forms of KS.

T-lymphocyte phenotypes and human leukocyte antigen-DR expression (HLA-DR) expression in skin lesions and draining lymph nodes of zoonotic leishmaniasis caused by Leishmania major were studied. T lymphocytes were the dominant cells in the lymphocytic preponderance type of lesion. They were lowest when the parasitized macrophage was the dominant cell or when the reaction consisted of an equal admixture of lymphocytes, macrophages and plasma cells. Both inducer/helper and suppressor/cytotoxic T cells were present in varying proportions. The latter were thought to be suppressor rather than cytotoxic cells. Their late appearance in the host reaction was believed to play a major role in halting the inflammatory response. Low levels of cells expressing the suppressor/cytotoxic phenotype and the continued presence of antigen in macrophages was associated with persistence of the lesions even when parasites were largely eliminated after specific antileishmanial therapy. Such lesions healed after local or systemic steroid therapy. The majority of cells in the dermal infiltrate were HLA-DR positive. Keratinocytes also expressed HLA-DR antigen.AM El-Hassan, R Kubba, YM Al-Gindan, AS Omer, MK Kutty, MBM Saeed, Lymphocyte

JAK2 p.V617F was the most prevalent mutation detected among patients in this study. Non-p.V617F JAK2 mutations were identified in exons 12 and 13 corresponding to recently reported mutations, except for the novel p.I540_N542delinsM.

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