Cases reported "Anemia"

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1/140. Spontaneous remission of anemia associated with a myelodysplastic syndrome with disease evolution into a myeloproliferative state.

    A red cell transfusion-dependent patient with a myelodysplastic syndrome had progression into a myeloproliferative state with thrombocytosis. At the same time, the patient became transfusion independent, and a subsequent bone marrow examination revealed a previously undetected loss of chromosome 7. The patient remains well with control of thrombocytosis by anagrelide therapy.
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keywords = bone
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2/140. Severe multisystemic hypersensitivity reaction to carbamazepine including dyserythropoietic anemia.

    OBJECTIVE: To report a case of multisystemic hypersensitivity reaction to carbamazepine. CASE SUMMARY: An 81-year-old white man was admitted to our hospital because of fever, morbilliform pruritic rash, and jaundice. Fifty days before admission he had taken carbamazepine 200 mg p.o. tid because of seizures. During the first few days following admission, a maculopapular rash progressed to generalized erythroderma with subsequent extensive skin exfoliation. After discontinuing carbamazepine the fever disappeared within 72 hours and hepatic function tests returned to normal within four days. Moreover, after admission the hemoglobin values gradually fell to 6.7 g/100 mL. A bone marrow aspirate showed hypercellularity with marked dyserythropoietic abnormalities, and the bone marrow biopsy showed large and diffused infiltration due to the presence of a low-grade small lymphocytic lymphoma. No specific therapy for the lymphoma was undertaken. The biochemical follow-up showed a total improvement of hemoglobin values. Eight months after drug discontinuation, the patient was asymptomatic; peripheral blood cell count and hemoglobin concentrations were persistently normal. DISCUSSION: To the best of our knowledge, this is the first published case report implicating carbamazepine as the cause of anemia associated with bone marrow hypercellularity and dyserythropoietic changes, instead of hypocellularity and reduction of erythroid precursors. An interesting point raised by our observation is the possible relation between carbamazepine intake and actual lymphoproliferative disease. The development of non-Hodgkin's lymphoma following carbamazepine treatment has been reported, with regression after the drug was discontinued. However, in our case, a bone marrow biopsy repeated eight months after drug discontinuation confirmed the diagnosis of low-grade lymphoma. CONCLUSIONS: This case report describes a severe multisystemic reaction, characterized by generalized erythroderma; and renal, hepatic, and bone marrow failure in a patient who started carbamazepine therapy 50 days beforehand.
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keywords = bone
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3/140. The use of monoclonal antibody R92F6 and polymerase chain reaction to confirm the presence of parvovirus B19 in bone marrow specimens of patients with acquired immunodeficiency syndrome.

    BACKGROUND: parvovirus B19 infection is a cause of chronic anemia and red cell aplasia in patients with acquired immunodeficiency syndrome (AIDS) and in other immunocompromised hosts. anemia in AIDS patients has a multifactorial etiology, with parvovirus B19 infection being an infrequent but nevertheless treatable cause. Therapy with intravenous immune globulin can result in rapid improvement of parvovirus-induced anemia. This treatment is expensive, therefore accurate and rapid confirmation of parvovirus infection is important in providing appropriate and cost-effective therapy. methods: Bone marrow samples from 2 AIDS patients with severe anemia and reticulocytopenia were studied. Bone marrow morphology and serologic studies were evaluated for parvovirus B19 infection. An immunohistochemical method using a monoclonal antibody, R92F6, to B19 capsid proteins was utilized on decalcified, B5-fixed, paraffin-embedded bone marrow biopsies. Bone marrow aspirate cells were examined by electron microscopy for evidence of viral particles. In addition, polymerase chain reaction (PCR) studies using a nested PCR assay to the parvovirus B19 viral genome were performed in a case for which fresh cells were available. RESULTS: Bone marrow findings included marked erythroid hypoplasia with characteristic giant pronormoblasts and intranuclear inclusions. Serologic studies were negative in one case, while the second case showed positive parvovirus B19 immunoglobulin m antibody. Immunohistochemical studies for parvovirus B19 were positive in both cases. The presence of intranuclear virions was demonstrated by electron microscopy and was confirmed by PCR analysis. Both patients were treated with intravenous immune globulin, and subsequent improvement was noted. CONCLUSIONS: Both immunohistochemistry and PCR studies on bone marrow specimens from AIDS patients with anemia are rapid and sensitive methods for the confirmation of parvovirus B19 infection. They are valuable tools, particularly when serologic studies are negative. When PCR is not available, immunohistochemical methods can be useful. The rapid confirmation of parvovirus B19 infection will allow for early and cost-effective therapy.
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4/140. Myelodysplastic syndrome that progressed to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormality: a case report.

    Myelodysplastic syndrome is a closely related group of acquired bone marrow disorders characterized by ineffective and dysplastic hematopoiesis. These clonal disorders frequently progress to acute leukemia. Acute myelomonocytic leukemia with eosinophilia is characterized by an increase in abnormal eosinophils in the bone marrow, relatively good clinical course and inv (16) chromosomal abnormality. We experienced one case of refractory anemia with excess blasts which progressed to refractory anemia with excess blasts in transformation and finally to acute myelomonocytic leukemia with eosinophilia showing peculiar chromosomal abnormalities of der (1;7).
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5/140. Hepatosplenomegalic lipidosis: what unless Gaucher? adult cholesteryl ester storage disease (CESD) with anemia, mesenteric lipodystrophy, increased plasma chitotriosidase activity and a homozygous lysosomal acid lipase -1 exon 8 splice junction mutation.

    A 36-year-old woman was admitted for hepatosplenomegaly and anemia. Bone marrow cytology showed "sea-blue histiocytes", vacuolated macrophages and plasma cells. As primary liver disease, malignancy or hematologic disorders were excluded, and plasma chitotriosidase activity was increased 27-fold over control, the presence of a lysosomal storage disease was suspected. Biochemical analysis of skin fibroblasts revealed normal glucocerebrosidase and sphingomyelinase activity, but lipid analysis showed a more than 15-fold accumulation of cholesterol esters within the cells. The activity of lysosomal acid lipase (LAL) in fibroblast homogenates was decreased to 12% of control subjects. Mutational analysis of the patient's blood showed the homozygous G-->A mutation at position -1 of the exon 8 splice donor site (E8SJM-allele) known for adult cholesteryl ester storage disease (CESD); the polymorphic background was that of the complex haplotype -6Thr, 2Gly, 894 G-->A. Based on clinical, laboratory, cytological and and biochemical findings, CESD can clearly be separated from other more frequent inherited lysosomal storage diseases, e.g. atypical forms of gaucher disease.
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ranking = 7.1295647457861
keywords = macrophage
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6/140. in vitro inhibition of hematopoiesis in a patient with systemic sclerosis treated with D-penicillamine.

    A 66-year-old Japanese woman with severe scleroderma developed anemia and thrombocytopenia due to D-penicillamine (D-Pen) treatment, although the leukopenia was not markedly severe. Cessation of D-Pen and the start of corticosteroid therapy were followed by recovery from bicytopenia. We examined the in vitro inhibition of the clonogenic capacity of bone marrow hematopoietic progenitor cells of this patient by D-Pen, and found strong inhibition in burst-forming unit-erythroid and colony-forming unit-megakaryocyte assays, but not in colony-forming unit-granulocyte/macrophage assays. These findings suggest that bicytopenia in this patient was caused by D-Pen and may be due to different sensitivities in the hematopoietic lineage.
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ranking = 8.1295647457861
keywords = macrophage, bone
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7/140. Behcet's disease associated with myelodysplastic syndrome: a case report.

    A rare case of Behcet's disease associated with myelodysplastic syndrome (MDS) is described. A 50-year-old Korean female suffering recurrent oral ulcer, genital ulcer, fatigue, arthralgia in both knees and fever was diagnosed as Behcet's disease. The findings of bone marrow aspirates were consistent with refractory anemia, a subtype of myelodysplastic syndrome. Chromosomal analysis of bone marrow cells revealed 46,XX,-8,-20, der(8)t(8;20)(p23;p10), der(8) t(8;20)(p23;q10)[30]. The chromosomal changes found in this patient were different from those of previous reports, which mostly revealed trisomy 8. If anemia, low reticulocyte count and dyspoietic cells are sustained in Behcet's disease, physicians should be alert to the possibility of MDS with aberration in chromosome 8 and perform a bone marrow study for the proper diagnosis and treatment of the disease. We presented a case of Behcet's disease associated with MDS, which is the first Korean case.
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keywords = bone
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8/140. parvovirus B19 infection in a human immunodeficiency virus-infected patient with anemia.

    anemia is generally attributed to zidovudine therapy in human immunodeficiency virus (hiv)-infected patients, although parvovirus B19 infection has been reported as a rare cause. We report on a 24-year-old homosexual man infected with hiv who presented with anemia. He had received aggressive daily antiretroviral therapy (zidovudine 600 mg, lamivudine 300 mg, and saquinavir 1,800 mg) for 2 years. At the time of admission, his CD4 count was 10 x 10(6) cells/L. A bone marrow aspirate smear showed a marked decrease in erythropoiesis and immunocytochemical staining for parvovirus B19 was positive. parvovirus B19 viral dna was detected in the peripheral blood using a polymerase chain reaction-based assay. Serologic studies were positive for parvovirus B19 immunoglobulin (Ig)M antibodies, but negative for IgG antibodies. The patient was treated with packed red blood cell transfusion. zidovudine was stopped and replaced with zalcitibine 2.25 mg daily after anemia occurred. He did not receive intravenous Ig therapy because of its cost. After discontinuation of zidovudine for 1 year, anemia persisted and the patient depended on regular blood transfusions to control the anemia. This case emphasizes that, in addition to drug-related causes, parvovirus B19 infection should be included in the differential diagnosis of chronic anemia in hiv-infected individuals.
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keywords = bone
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9/140. Diffuse angiodysplasia of the upper gastrointestinal tract in a patient with hypertrophic obstructive cardiomyopathy.

    A 64-year-old woman with a known history of hypertrophic obstructive cardiomyopathy presented with severe anemia of unknown origin. She had also suffered from repeated episodes of upper gastrointestinal bleeding for the previous 3 years. Despite bone marrow examination and panendoscopic and angiographic studies, the origin of anemia remained undefined until a small bleeding site was found during a duodenoscopic examination. The lesion proved to be angiodysplasia. This case report is interesting in that angiodysplasia elicited gastrointestinal bleeding and was the cause of anemia. In the international literature, there are very few reported cases of bleeding from gastrointestinal angiodysplasia in association with subvalvular aortic obstruction.
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keywords = bone
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10/140. Acute hepatitis in adult Still's disease apparently resulting from oral iron substitution--a case report.

    The authors report a case of a young woman with adult-onset Still's disease (AOSD) with massive hyperferritinaemia who developed acute florid hepatitis with intraparenchymatous histiocytic infiltration following oral iron substitution for presumed iron deficiency, which settled on withdrawal of the iron. This suggests that the iron exacerbated the macrophage hyperactivity which is presumed to be present in AOSD. Oral iron substitution in the acute phase of this disease may be inadvisable.
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ranking = 7.1295647457861
keywords = macrophage
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