Cases reported "Bone Marrow Diseases"

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1/53. Shwachman-diamond syndrome: early bone marrow transplantation in a high risk patient and new clues to pathogenesis.

    Shwachman-diamond syndrome (SDS) is an autosomal recessive disorder characterised by exocrine pancreas insufficiency, metaphyseal dysostosis and bone marrow dysfunction. Recurrent severe bacterial infections and susceptibility to leukaemia are the major causes of morbidity and mortality occurring preferentially in patients with pancytopenia and features of myelodysplasia. Here we report a patient with SDS leading to recurrent bacterial infections and a deteriorating condition since early infancy. Extensive investigations disclosed severe pancytopenia, myelodysplasia and a clonal cytogenetic abnormality, inv(14)(q11q32), as risk factors of leukaemic transformation. He therefore underwent allogeneic geno-identical bone marrow transplantation which resulted in correction of all haematological and immunological abnormalities within an 18-month follow up period.Conclusion bone marrow transplantation may be considered early as a valuable treatment option especially in high risk Schwachman-diamond syndrome patients anticipating malignant transformation, life-threatening severe infections or further organ damage.
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2/53. Erythroleukemia-like syndrome due to busulfan toxicity in polycythemia vera.

    Over a 19-year period, a patient with polycythemia vera who had undergone a splenectomy received six courses of busulfan for recurrent thrombocytosis. The total dose of busulfan given for the sixth course was greater than that used for the previous ones. Severe pancytopenia followed, which persisted for 4 months. During this period there was marked erythroid hyperplasia in the bone marrow with striking dyserythropoiesis; PAS-positive red cell precursors, as well as moderate numbers of circulating normoblasts and evidence of chronic and acute hemolysis, were present. All of these findings reverted to normal without therapy, and the polycythemic state eventually recurred. These events are interpreted as an unusual marrow reaction following busulfan overdosage rather than a transient erythroleukemia.
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3/53. Reversal of bone marrow hypoplasia in anorexia nervosa: case report.

    BACKGROUND: Bone marrow hypoplasia seems to be uncommon in anorexia nervosa. This marrow abnormality is rapidly reversible with intensive nutritional rehabilitation. The patient described in this case report had anorexia nervosa. METHOD: She presented with complete serous atrophy of the bone marrow associated with pancytopenia. RESULTS: She was cured with recombinant human erythropoietin administered subcutaneously. After 25, days, the hematological situation was normalized. DISCUSSION: The possible advantage and practical implications of the administration of erythropoietin are discussed.
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4/53. Successful unrelated bone marrow transplantation for Shwachman-diamond syndrome.

    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.
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5/53. Massive plasmocytosis due to methimazole-induced bone marrow toxicity.

    pancytopenia is a rare complication of the thionamide therapy reported secondary to aplastic anemia, the bone marrow being invariably hypocellular. We present a case of a 16-year-old female with Graves' disease who presented with massive bone marrow plasmocytosis mimicking multiple myeloma. The patient had already been on methimazole for a month when she was admitted to the Pediatric Unit with the diagnosis of sepsis. CBC revealed pancytopenia. Bone marrow aspirations showed hypocellular-normocellular bone marrow, 98% of plasma cells. At that time, MMI was discontinued and the patient was started on broad-spectrum antibiotics, dexamethasone, and G-CSF. Bone marrow aspiration day 4 still showed hypo-normocellular marrow, with remaining 6% plasma cells. Myeloma screen was negative; ANC >1,000 at day 7, platelets >50,000 at day 24. Twenty-four months after patient's discharge, her clinical condition, CBC, and bone marrow remained normal. To our knowledge this is the first report of pancytopenia due to MMI, where the usual hypoplasia found is replaced by massive plasmocytosis.
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6/53. ehrlichiosis infection in a 5-year-old boy with neutropenia, anemia, thrombocytopenia, and hepatosplenomegaly.

    ehrlichiosis should be considered in the differential diagnosis of any patient with recent fever, pancytopenia, hepatosplenomegaly, and history of tick exposure. We present a previously healthy 5-year-old boy who was referred to the hematology-Oncology Clinic to consider a bone marrow etiologic process after his pediatrician discovered progressive neutropenia, anemia, thrombocytopenia, and hepatosplenomegaly accompanied by 2 days of fever. Bone marrow aspirate and biopsy were nonrevealing. Because of the history of a recent tick bite, a diagnosis of ehrlichiosis infection was considered and ultimately confirmed by IgG-specific serum testing. The patient's fever was treated symptomatically with acetaminophen, and symptoms resolved on their own without intervention. ehrlichiosis is a tick-borne infection that occurs throughout the spring and summer, often causing findings that mimic a malignancy or serious hematologic disorder. The diagnosis should be considered in any person living in tick-infested areas and can be confirmed by polymerase chain reaction or serum antibody titers. Treatment with doxycycline can lead to rapid clinical improvement if the diagnosis is made early.
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7/53. Severe reversible bone marrow suppression induced by Selaginella doederleinii.

    Case Report: We report a 52-year-old female patient with cholangiocarcinoma who developed severe bone marrow suppression after taking Selaginella doederleinii as an alternative anticancer treatment. She developed severe pancytopenia with initial presentations of skin ecchymosis, itching, and gum bleeding 2 weeks after taking Selaginella doederleinii daily. Bone marrow smear and biopsy showed severe hypocellularity with no malignant cell infiltration. Approximately 1 week after stopping Selaginella doederleinii, her hemogram returned to previous levels. Selaginella doederleinii, a popular anticancer herb, may contain an as yet unidentified substance that contributes to reversible bone marrow suppression.
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8/53. Bone marrow hypoplasia during brucella infection.

    pancytopenia, although mainly reported in adults, has also been described in children with brucellosis. However, bone marrow hypoplasia is a rare feature of the infection. An 11-year-old boy was admitted with fever, vomiting, and abdominal pain of 10 days' duration. On physical examination, pallor and high fever were detected in the absence of lymphadenopathy and hepatosplenomegaly. His hemoglobin was 8.6 g/dL, white blood cell count 1,100/mm(3), neutrophil count 500/mm(3), platelets 56,000/mm(3), and reticulocytes 0.1%. Hypocellular bone marrow was found by aspiration, and bone marrow biopsy revealed hypocellularity. The agglutination titer was greater than 1/640. trimethoprim/sulfamethoxazole was prescribed. His fever subsided and pancytopenia subsequently improved. pancytopenia associated with brucellosis is attributed to hypersplenism, hemophagocytosis, and granulomatous lesions of the bone marrow, which is usually hypercellular. Bone marrow hypoplasia is rarely reported and should be kept in mind in the etiology of aplastic anemia in a country where brucellosis is frequently encountered.
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9/53. Bone marrow necrosis in paediatric patients.

    necrosis of bone marrow is a very rare condition with a prevalence ranging from 0.15 to 7 percent. It is mostly not compatible with life. We diagnosed four cases of necrotic marrow out of 830 bone marrow aspirations done in last five years of time. All were paediatric patients having peripheral pancytopenia. Three out of four patients expired within one month of diagnosis.
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10/53. Severe refractory sarcoidosis in a 64-year-old man with persistent leucopenia.

    We present a case of bone marrow granulomas in a 64-year-old West Indian man who presented with severe leucopenia, anaemia, thrombocytopenia, hepatosplenomegaly, hypercalcaemia, hypercalciuria, elevated angiotensin converting enzyme level and reticulo-nodular shadows on chest X-ray. Bone marrow biopsy revealed numerous non-caseating epithelioid granulomas. A diagnosis of sarcoidosis was made and he was treated with prednisolone 60 mg daily for four weeks and the dose was subsequently reduced to 30 mg daily. Eight months follow-up revealed persistent pancytopenia. Bone marrow granulomas are rare and, when they occur, sarcoidosis is an uncommon aetiology. This case illustrates that severe leucopenia may occur in sarcoidosis and may present therapeutic difficulties.
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