31/523. Disseminated histoplasmosis in a non-immunocompromised child. We describe a 2 year-old non-immunocompromised girl with disseminated histoplasmosis who presented with a 2-month history of fever and bloody diarrhoea. On presentation, she was severely wasted and anaemic. There were gross hepatosplenomegaly and multiple lymphadenopathy. A septic screen was negative. A subsequent stool culture isolated salmonella enteriditis. Serial Widal-Weil Felix (WWF) titres showed serological response after 2 weeks of ceftriaxone. However, she continued to have spiking fever, bloody diarrhoea and weight loss. She developed pancytopaenia and disseminated intravascular coagulation. A bone marrow aspirate and trephine, and lymph node biopsy showed the presence of histoplasma capsulatum, confirmed by Gomori-methenamine silver staining. She responded to intravenous amphotericin b followed by fluconazole (intravenous then oral) for 6 months after discharge. Human Immunodeficiency Virus screening tests were negative. Complement and immunoglobulin levels were normal. T and B enumeration tests showed gross leucopaenia with very low T cell function with defective phagocytic function. A repeat T and B cell enumeration test and phagocytic function tests done 3 months later were normal. ( info) |
32/523. Severe pancytopenia after leflunomide in rheumatoid arthritis. Leflunomide is a novel drug recently introduced for treatment of rheumatoid arthritis as a DMARD (disease-modifying antirheumatic drug). So far, leflunomide has not been associated with severe bone marrow toxicity and pancytopenia. We report of a 62-year-old woman with a 14-year history of rheumatoid arthritis with bone marrow toxicity and reversible pancytopenia developing after treatment with leflunomide. ( info) |
| patients who develop cholestatic jaundice during chronic total parenteral nutrition (TPN) can develop significant hematologic complications due to hypocupremia if copper supplementation is withheld. A 36-year-old female with short bowel syndrome developed progressive liver dysfunction 6 months after initiation of TPN. trace elements were omitted from her TPN because of cholestasis and persistent hyperbilirubinemia. Despite chronic diarrhea, absorption of some dietary copper was anticipated from her oral diet. Fifteen months later, the patient became red cell transfusion dependent, and her neutrophil and platelet counts steadily declined. After 19 months of receiving TPN without trace elements, her serum copper level was 25 microLg/dL (normal: 70 to 155 microg/dL). Provision of trace elements for 2 months was associated with increased serum copper, neutrophil and platelet counts and independence from red cell transfusions. When the serum copper level reached 186 microg/dL, copper supplementation was discontinued. Over the next 3 months, serum copper level fell to 10 microg/dL, neutrophil and platelet counts fell precipitously, and red cell transfusions were resumed. Once again, copper, neutrophil and platelet levels promptly rebounded with parenteral copper supplementation. Although anemia and neutropenia are well-recognized hematologic consequences of copper deficiency, thrombocytopenia rarely has been reported. This is the first report of pancytopenia secondary to TPN-related copper deficiency in which the association was confirmed when hypocupremia recurred. ( info) |
34/523. growth failure, intracranial calcifications, acquired pancytopenia, and unusual humoral immunodeficiency: a genetic syndrome? We report on two children who may represent a novel syndrome consisting of a deficiency of immunoglobulin-bearing B lymphocytes and serum antibody, deficient intrauterine and/or postnatal growth, intracranial calcifications, and acquired pancytopenia. Poor growth, intracranial calcifications, developmental delay, and hematological abnormalities are common manifestations of congenital infection. However, humoral immunodeficiency is not characteristic in these infections, and no infection was found on extensive evaluation. Rare genetic syndromes may mimic intrauterine infections and may also include immunodeficiency. However the children reported here lack important characteristics or share distinctive manifestations not described in these disorders. Infants presenting with apparent congenital infections in whom a specific infectious cause cannot be identified should be followed carefully with immunological evaluations since this disorder may be progressive and considerable morbidity is attributable to hematological and immunological manifestations. ( info) |
35/523. Aplastic anemia: current concepts and dental management. Aplastic anemia (AA) is a rare blood dyscrasia in which the peripheral blood cells are decreased because of bone marrow failure. The clinical course reflects the severity of pancytopenia and is unpredictable for the individual. hemorrhage and infection remain the major threats to these patients. Recent advances in transfusion medicine, infection management, bone marrow transplantation, and immunosuppressive therapy have improved survival of patients with AA. oral manifestations of AA are common and may have serious sequelae. Two cases of acute periodontal infection associated with AA are presented. Dental management guidelines are presented in the context of interdisciplinary care. ( info) |
36/523. Successful non-surgical treatment of disseminated polymicrobial fungal infection in a patient with pancytopenia and graft-versus-host disease. Invasive fungal infections after bone marrow transplantation have an extremely poor prognosis. Surgical excision in combination with antifungal therapy is considered necessary for treatment, especially for central nervous system (CNS) infection. We describe successful medical management with lipid complex amphotericin b (ABLC) and itraconazole, without surgical excision, of disseminated fungal infection involving the lungs and CNS in a patient with pancytopenia and graft-versus-host disease. ( info) |
37/523. PML/RAR alpha( ) hypergranular acute promyelocytic leukemia (M3) developing into an M3 acute myelocytic leukemia without PML/RAR alpha. The case of an adult with PML/RARalpha( ) hypergranular acute promyelocytic leukemia (M3) that evolved into a rapidly fatal M3 acute myelocytic leukemia without PML/RARalpha after a complete and molecular remission had been achieved, is presented. Only 7 such cases have been published in the literature. The possible origin of this either leukemic relapse or secondary malignancy is briefly discussed, focusing on the fact that this diagnosis could only be defined by adequate molecular biology studies in the leukemic cells. ( info) |
38/523. Rare side-effects of fenofibrate. Fibrates are widely prescribed as hypolipidemic drugs and are considered as safe. We report the case of a 69 year-old woman who probably developed a major allergic reaction following a fenofibrate prescription (generic form) of 300 mg per day. Clinical features included asthenia, hyperthermia (40.5 degrees C) and slight muscular pain. Biological abnormalities were mildly elevated muscular enzymes and pancytopenia rapidly developed. All bacteriologic, virologic, immune and radiologic investigations were normal. Evolution was spontaneously favorable with fenofibrate withdrawal. This is the first reported case of major fever and pancytopenia following a fenofibrate prescription. Adverse effects of fenofibrate are briefly reviewed and their usual favorable outcomes following drug removal are outlined. ( info) |
| A 5-year-old boy with Shwachman-diamond syndrome underwent unrelated HLA-identical bone marrow transplantation for severe pancytopenia. Conditioning was with busulfan, thiotepa and cyclophosphamide plus rabbit anti-lymphocyte serum. Engraftment for neutrophils and platelets was observed on days 18 and 41, respectively. Transplant-related side-effects were mild and transient. After a follow-up of 32 months, the patient is alive and enjoys a normal life, off any immunosuppressives. Immunological and hematological reconstitution is complete while other phenotypic characteristics (pancreatic insufficiency, short stature, femur dysostosis) are stable. Although experience in this field is scarce, we speculate that bone marrow failure in Shwachman-diamond syndrome (even if not linked to the appearance of clonal disorders or leukemic transformation) is an indication for bone marrow transplantation and may be associated with a better outcome. ( info) |
40/523. Parovirus B19 infection in an hiv-infected patient with febrile pancytopenia and acute hepatitis. The case of a 34-year-old male patient with hiv infection referred for severe febrile pancytopenia is reported. Clinical and laboratory evaluations revealed acute hepatitis b infection and concomitant parvovirus B19 infection. The patient died just before treatment with immune globulin was to be administered. parvovirus B19 has been found to cause a wide variety of hematologic disorders such as neutropenia, thrombocytopenia, pancytopenia, and hemophagocytic syndrome. The role of parvovirus B19 in the pathogenesis of bone marrow or liver involvement is briefly discussed. ( info) |