[A new biological sign of Waldenstrom's disease: increase in alkaline phosphatase activity of the granulocytes].
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Biomedical subjects
Publications and source records attributed to A Broustet.
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The syndrome of CD8 hyperlymphocytosis with neutropenia is a heterogeneous disorder ranging from reactive benign state to neoplastic pathology. The prognosis for LGL (Large Granular Lymphocyte) leukemia depends likely on its phenotype:-NK phenotype, extremely poor prognosis and rapidly fatal-T phenotype (CD8+), chronic disease with slow progression. Here, we report four cases of CD8+ hyperlymphocytosis with neutropenia, which are CD2+/-, CD3+, CD4-, CD8+, CD16-, CD56+/-, CD57+ phenotype. These lymphocytic proliferations were associated with clonal rearrangement of T-cell receptor b gene. In two cases, characteristic blood hyperlymphocytosis appeared only after splenectomy, but retrospective bone marrow analysis showed that the CD8+, CD57+ lymphocyte proliferation previously existed. These lymphocytes had a low natural killer activity against K562 cell line. HTLV1 proviral sequence was not integrated in leukemic cell DNA. This monoclonal pathology has a chronic clinical course, with a thirteen year evolution in one case. Splenectomy did not correct neutropenia but allowed the control of hemolytic anemia and auto-immune thrombocytopenia in one case.
We report 12 cases of leucocytoclastic vasculities associated with myelocytic (7 cases) or lymphocytic (5 cases) blood diseases. The clinical features, laboratory abnormalities and pathological findings are presented. In all cases a drug-induced and/or infective origin could be ruled out, and patients with cryoglobulinaemia were excluded. As in other cases found in the literature, in some patients vasculitis was present several weeks or months before the blood disease was discovered, while in others both conditions developed simultaneously or vasculitis appeared in the course of the blood disease. None of these three possibilities seemed to make the prognosis worse. The pathogenesis of the vasculitis-malignant blood disease association is uncertain. Immune complexes of tumoral origin undoubtedly have some responsibility, but some deficiency of phagocytic cells or chemotactic factors also play a role. Cases such as ours enable the significance of vasculitis to be understood when it occurs in a patient with malignant blood disease, but above all they should prompt investigations for malignant blood disease in patients with cutaneous and/or systemic vasculitis, as is being done in patients with pyoderma gangrenosum or Sweet's syndrome.
The authors report a case of lymphomatoid papulosis with an ultrastructural study, and review 84 cases which they found in the literature. They recall the clinical and histological signs and the course of this disease which usually has a benign prognosis. The classification and relationship with varioliform parapsoriasis of Mucha-Habermann and the hematodermias remain debated.
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Although primitive plasma cells leukaemia is uncommon, it still stands in a particular place among malignant lymphoplasmocytary syndroms. It borrows from multiple myeloma and acute leukaemia some of its clinical and biological manifestations. Its course is quickly adverse, its treatment ineffective and the average life expectation is three months.
A progressive paraplegia appeared in a 54 years old patient during a chronic eosinophilic leukemia. Myelography revealed a block of the spinal canal at T3. Laminectomy discovered an extramedullary hematopoiesis in the epidural space. The published literature is reviewed.