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Abstract:Hydroxyurea is an hydroxylated urea derivative used in many myeloproliferative disorders. Many, but unusual cutaneous disorders are related after its prolonged use. Their pathogenesis is not clear, but it is suggested that there is direct toxicity of the drug on the skin. We described a white, 75-year old man with diagnosis of Polycythemia Vera that in 11 years of treatment developed many cutaneous lesions: skin hyperpigmentation, atrophic lesions on forearms, longitudinal melanonychia of 20 nails, right forearm ulcer, cutaneous xerosis, ichthyosis and auricular spinocellular carcinoma. At this moment, the literature reports describe little diversity of lesions in affected patients. Keywords: Hydroxyurea; Polycythemia vera; Skin manifestations Resumo: A hidroxiureia é um derivado hidroxilado da ureia utilizado em diversas desordens hematológi-cas. Inúmeras alterações cutâneas, porém raras, são relatadas após seu uso prolongado. A patogênese das mesmas não está bem esclarecida, porém, sugere-se que a droga tenha uma ação tóxica direta sobre a pele. Descrevemos um homem de 75 anos, branco, com diagnóstico de Policitemia Vera que, ao longo de 11 anos de tratamento com hidroxiureia, evoluiu com várias lesões cutâneas: hiperpigmentação da pele, lesões atró-ficas em antebraços, melanoníquia longitudinal das 20 unhas, úlcera em antebraço direito, xerose cutânea, ictiose em pernas e carcinoma espinocelular no pavilhão auricular direito. Até o momento, os relatos na literatura descrevem pouca diversidade de lesões nos pacientes acometidos.

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Hemophilia A is a blood clotting disorder characterized by a mutation of the factor VIII (FVIII) gene, leading to a deficiency in FVIII. It is the most common type of hemophilia. Inheritance is X-linked; hence males are affected while females are carriers or very rarely display a mild phenotype. 1:10,000 males are affected. We describe a child with a rare association of congenital hemophilia A and acquired severe aplastic anemia (SAA) who was successfully treated with allogeneic bone marrow transplantation (allo-BMT). A 6-year old male, SG, was diagnosed with hemophilia A (FVIII 3%) at 6-month old when he presented with right knee hemarthroses and psoas muscle hematoma. His maternal grandfather was hemophiliac. Since his diagnose, FVIII was infused on average once a month, generally due to hemarthroses and bleeding secondary to trauma. In July 2004, pt presented with progressive pancytopenia. In January 2005 CBC showed: Hb 7g/dl, Ht 22%, WBC 1000/μl (absolute neutrophil count 250/μl) and PLT 23,000/μl. Bone marrow aspirate and biopsy were consistent with SAA. DEB test was negative. Since then he was poly-transfused with PLT and RBC due to increased bleeding tendency, especially hematoma and petechiae. In July 2005 he underwent an allo-BMT from his HLA-identical non-hemophiliac 5-year old brother. For catheter implantation pt received PLT transfusion to achieve a PLT level greater than 50,000/mm3 and 60 units/kg of FVIII to achieve an initial level of 100% followed by 30 units/kg every 12 hours to maintain it continuously at greater than 50% for 3 days. Conditioning consisted of cyclophosphamide 50mg/day from D-5 to D-2 and thymoglobulin 5mg/kg from D-3 to D-1. Cyclosporine and a short course of methotrexate were given for GVHD prophylaxis. 3,5x108 mononuclear cells/kg were infused. Allo-BMT was uneventful. Neutrophil engrafted on D+15 and megakaryocyte (>50,000/mm3) on D+38. During the transplant PLT were kept at greater than 30,000/mm3 with PLT transfusion every 2 days. FVIII was infused when there was more than 2 cm increase in the knee perimeter or any other abnormal bleeding, especially at the catheter implantation site. 6 months after allo-BMT the child is well, with a normal CBC and coagulation tests similar to pre-BMT. Hemophilia and AA association is rare, and to our knowledge, this is the first allo-BMT in a hemophiliac patient to be reported.

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Background: B-cell chronic lymphocytic leukemia (B-CLL) is characterized by the progressive accumulation of a neoplastic clone consisting of CD5/CD19/CD23/surface immunoglobulin cells. Despite this homogeneity in phenotype, B-CLL can follow either an indolent or a progressive course. CD38 is a transmembrane glycoprotein expressed on the surface of leukemic cells in a significant percentage of patients with B-CLL. Recent studies suggest that CD38 expression in CLL is a reliable prognostic marker, leading to an unfavorable clinical course with a more advanced stage of disease, poor responsiveness to chemotherapy and a shorter survival rate, along with others markers like ZAP-70 that reflect more accurately the mutagenic status of VH gene region, unfortunately not yet available in clinical practice routinely. Objectives: To assess the association of CD38+ expression with clinical and laboratory parameters at diagnosis in patients with B-CLL. Patients and Methods: CD38 expression was analyzed in 64 unselected newly diagnosed B-CLL patients from January 2003 to May 2005 seen at the Hematology Center of Pernambuco-Brazil (HEMOPE). According to Damle et al. patients with 30% or more B cells expressing CD38 were considered positive(CD38+), and those with less than 30% were considered negative(CD38−). Various patient characteristics were studied including age, sex, Binet stage, hemoglobin, ß2 microglobulin and lactate dehydrogenase levels in the serum. Statistical differences between each group (CD38+ vs CD38−) were analyzed using χ2 tests for categorical variables and Student’s t-tests for continuous variables. Results: Thirty-six patients (56.25%) were CD38+ and 28 (43.75%) were CD38-. Their median age at diagnosis was 64 years (range, 39–83) and the male to female ratio was 1.46:1. There were no differences between age, sex, hemoglobin and ß2 microglobulin levels in the CD38+ and CD38- groups. ß2 microglobulin level, however, was not available for assessment in all patients. Lactate dehydrogenase level was significantly higher in the CD38+ group (p=0.007). It was observed a trend toward a higher Binet stage (B or C) in the CD38+ compared with CD38- group (63.4 vs. 36.6% respectively), although not statistically significant (p=0.09) possibly because of the small number of patients studied. Conclusion: CD38 expression was associated with a higher lactate dehydrogenase level and a tendency for more aggressive clinical stage. CD38 evaluation is a measurable biological parameter that is not as subjective as the evaluation of some clinical parameters and should be considered a stantard clinical test for newly diagnosed B-CLL patients.