Histiocytic medullary reticulosis.
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Biomedical subjects
Publications and source records attributed to F Aversa.
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Intracytoplasmic lysozyme was studied by the peroxidase antiperoxidase (PAP) and protein A-peroxidase methods in 130 cases of various myeloproliferative and lymphoproliferative disorders and 21 lymph nodes and bone marrow metastases from solid primary tumors. This marker, which can be identified in formalin or Zenker-fixed tissues, as well as in peripheral blood and bone marrow smears, proved useful to distinguish malignant myeloid and histiocytic tumors from malignant lymphoid and undifferentiated epithelial metastases. The diagnostic application of these findings are discussed.
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An unusual case of multiple myeloma with extramedullary dissemination and co-existing systemic non-necrotic granulomas is described. Immunoperoxidase staining revealed that both lymph node and bone marrow infiltrating plasma cells contained the same IgG-lambda type paraprotein, and that the granuloma epithelioid histiocytes contained intracytoplasmic lysozyme. As there was no evidence of either systemic infection or sarcoidosis, the non-necrotic granulomas may represent the host's response to the myeloma, not unlike the reaction observed in Hodgkin's disease or solid tumours. A causal association cannot, however, be entirely excluded.
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Ultrastructural characterization and localization of intracellular immunoglobulin in peripheral blood mononuclear cells from a patient with hairy cell leukemia were studied by the direct immunoperoxidase technique. Approximately 20--30% of hairy cells stained positive for gamma-heavy and K-light chains, and this staining was limited to certain rough endoplasmic reticulum profiles. These ultrastructural findings can be considered an additional argument for the B cell origin of hairy cells.
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The administration of trimethoprim (TMP)--a diamino benzylpyrimidine compound which binds very tightly the bacterial dihydrofolate reductase--was accompanied by the appearance of measurable levels of dihydrofolate reductase in peripheral leukocytes from patients with nonhematological diseases. In all instances, enzyme activity rose rapidly between the fourth and eighth day after TMP. The time course of the rise and fall of dihydrofolate activity approaches cellular life span and is similar to that obtained after methotrexate or triamterene administration. Dihydrofolate reductases, partially purified from leukocytes of patients treated with TMP, bone marrow and leukemic leukocytes, had simila molecular weights, pH optima, Ki of inhibitor (methotrexate); they were stimulated to the same degree by KCl and urea. Electrophoresis of the enzyme on cellulose acetate strip resulted in the separation of two enzymatically active protein components. No differences in the electrophoretic behavior of the three blood cell enzymes were noted. The findings noted above are consistent with the suggestion that the observed rise in dihydrofolate reductase activity is a quantitative one. Moreover, the effect of TMP in vivo is discussed in comparison with the currently held hypothesis for methotrexate action (stabilization by the drug of a previously synthetized enzyme).
A 78-year old man is presented with a characteristic case history and physical and laboratory findings typical of angio-immunoblastic lymphadenopathy with dysproteinemia (AILD). The disease had an acute onset with constitutional symptoms, generalized lymphadenopathy and hepato-splenomegaly. The presence of a large pleural effusion was of particular interest. Histologically the distinctive feature was a pronounced proliferation of small blood vessels and immunoblasts in the lymph nodes. Management of patient with AILD is problematic. Since AILD is a non-neoplastic process, symptomatic treatment with small doses of steroids, if necessary, would seem to be the best therapeutic approach. However, in our patient, as well as in other cases reported in the literature, a rapid and long standing (18 months so far in our case) complete remission was obtained with a short cycle of chemiotherapy.
In a prospective randomized trial parenteral trimethoprim/sulphamethoxazole was added to amikacin plus piperacillin in order to compare triple-drug antibiotic combination with a standard regimen as empiric therapy of fever in patients with granulocytopenia. One hundred and sixty-one episodes were evaluated; 74 episodes with amikacin plus piperacillin and 87 episodes with amikacin plus piperacillin plus trimethoprim/sulphamethoxazole. The overall response to therapy (63% vs. 84%) as well as the response of microbiologically documented infections (60% vs. 82%) was significantly better in patients treated with the triple-drug combination (p less than 0.05). However, no statistically significant differences in response to antibiotics at different infection sites or with regard to any single pathogen was found between the two groups. Trimethoprim/sulphamethoxazole seemed to be responsible for additional toxicity (nausea and vomiting) when added to amikacin plus piperacillin, but these side-effects were clearly related to the rate of infusion of trimethoprim/sulphamethoxazole. The findings of this study support the use of a three-drug versus a two-drug combination as empiric antibiotic regimen in febrile granulocytopenic patients.
BACKGROUND: Nearly 40% of patients requiring a hematopoietic stem cell transplant lack a suitable donor. However, virtually all these patients have a potential family donor with whom they share one HLA haplotype. METHODS: We report the rationale for making hematopoietic stem cell transplantation from haploidentical related donors feasible, as well as the method followed to achieve this. Two studies are reported, designed to overcome the problem of rejection and graft-versus-host disease after haploidentical stem cell transplantation. We describe how our total body irradiation-based, highly immuno- and myelosuppressive conditioning regimens were developed and how they have been modified over the years in an attempt to improve the clinical outcome of high-risk acute leukemia patients receiving large numbers of extensively T-cell-depleted hematopoietic stem cell transplantations from full-haplotype mismatched family donors. RESULTS: A high engraftment rate and an extremely low incidence of graft-versus-host disease were obtained. Modifications of the pretransplant schedules allowed the reduction of transplant-related toxicity. CONCLUSIONS: The main obstacles that limited the use of haploidentical stem cell transplantation have been overcome. The procedure is now a reality that should be recommended in high-risk acute leukemia patients who do not have a suitable matched donor.
Severe combined immunodeficiency disease (SCID) with adenosine deaminase (ADA) deficiency is a genetic autosomic recessive disorder with profound impairment of T-cell function, invariably complicated by fatal infections. The absence of ADA-enzyme and the accumulation of deoxy-ATP, with toxic effects on the T-lymphocytes is the common feature of this disease. As a consequence, lymphoid precursors failure to develop into mature T-cells, resulting in absolute lymphopenia and atrophy of the thymus. Bone marrow transplantation from an HLA-identical donor is considered the treatment of choice for this disease. We describe the case of a 1-month-old child with ADA deficiency SCID who underwent bone marrow transplantation (BMT) using paternal haploidentical, lectin-separated marrow, as a source of hemopoietic stem cells.
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