Composite mantle-cell lymphoma and classical Hodgkin lymphoma.
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Biomedical subjects
Publications and source records attributed to J B Houghton.
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Papuloerythroderma of Ofuji (PEO) is an uncommon skin condition most commonly described in Japan. The aetiology of PEO is unknown and treatment may be difficult. There are several reports of an association between PEO and malignancy, most particularly with T-and B-cell lymphomas. We describe PEO complicated by acute myeloid leukaemia occurring in a 62-year-old man. This association has not been reported previously.
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Haemophagocytic lymphohistiocytosis (HLH) is a disease characterised by peripheral blood pancytopenia secondary to haemophagocytosis of formed blood cells by activated histiocytes. The demonstration of haemophagocytosis may be difficult and the diagnosis may require repeated tissue sampling (including bone marrow, cerebrospinal fluid, lymph nodes, spleen, and liver) and the demonstration of associated clinical or laboratory features. This report describes pronounced dyserythropoiesis in the bone marrow aspirates in four patients with HLH, including familial and secondary cases. In three patients, bone marrow haemophagocytosis was inconspicuous or absent, and the prominent dyserythropoiesis may have suggested an alternative diagnosis. The dyserythropoiesis observed should be added to the constellation of clinical and laboratory features associated with HLH.
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Extramedullary cutaneous plasmacytoma is an uncommon observation in clinical practice. In most of the cases it is usually an extension of underlying medullary plasmacytoma. This case report describes a patient with IgD myeloma who went into plateau phase following standard intravenous chemotherapy. After more than a year in plateau phase, he developed multiple fleshy nodular lesions in the skin. Biopsy of one of the lesions revealed relapse of myeloma in the skin. His IgD paraprotein remained in plateau phase and the bone marrow showed only 1% plasma cells. The patient has been treated with systemic intravenous chemotherapy followed by local radiotherapy.
Seventeen cases of infections due to Cunninghamella species have been reported worldwide in humans, and there have been only three survivors. We report a case of paranasal sinusitis due to Cunninghamella bertholletiae in an elderly patient who had diabetes mellitus and myelodysplasia. After receiving 7 weeks of therapy with deoxycholate amphotericin B (44 mg/kg or a total of 3 g) and rifampin, the patient was cured and did not have to undergo radical surgery.
A 51 year old man with Waldenström's macroglobulinaemia presented with a malabsorptive syndrome related to extensive small bowel lymphangiectasia caused by immunoglobulin accumulation. The patient's plasma had strong lupus anticoagulant activity and the IgM lambda paraprotein displayed specificity for the negatively charged phospholipids phosphatidyl serine and phosphatidyl inositol, as well as the neutral phosphatidic acid. Despite treatment for the macroglobulinaemia the patient died and at necropsy was found to have myocardial ischaemia and segmental infarcts in the spleen and kidney. The coexistence of these relatively rare findings suggests a possible association between Waldenström's macroglobulinaemia with gastrointestinal manifestations and paraprotein specificity for phospholipid.
The authors report a case of Castleman's disease in which the major clinical problems have been caused by severe thrombocytopenia and neutropenia. The cytopenias were caused by autoimmune destruction of mature cells as demonstrated by bone marrow findings and cell-specific antibody assays. There also was evidence of red cell autoantibody production but no overt hemolysis. The thrombocytopenia currently is controlled by intermittent vincristine, having proved refractory to steroids, splenectomy, and danazol and only partially responsive to high dose intravenous immunoglobulin. The neutropenia showed temporary improvement with immunoglobulin but has been resistant to all other therapy. This case provides further evidence for disordered immune regulation in Castleman's disease.
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We describe an immunofluorescence test to detect neutrophil-specific antibodies using neutrophils fixed to a slide and frozen prior to use. In titration studies using NA1, NA2 and NB1 antisera the technique was less sensitive than the standard immunofluorescence test but did enable slides to be stored for at least 5 months with no loss of NA1, NA2 and NB1 antigens. This technique can be used as a simple alternative to neutrophil cytoplasts for the identification of neutrophil antibodies. However, its use as a screening test in neutrophil serology is limited by the occurrence of false negative reactions with weakly reactive antisera.
Haemopoietic dysplasia is a condition which often precedes the development of acute non-lymphocytic leukaemia. Before this event, however, patients are at risk from severe infections even in the absence of neutropenia. This paper describes 3 patients with haemopoietic dysplasias in whom neutrophil microbicidal activity was deficient in vitro. The important abnormality appeared to be defective release of myeloperoxidase into the phagocytic vacuole. Two of these patients suffered from numerous baterial infections.
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