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Hypocomplementemia and hematological abnormalities in immunoblastic lymphadenopathy and immunoblastic lymphadenopathy-like T cell lymphoma. 1996

T Higuchi, and H Mori, and H Niikura, and M Omine
Division of Hematology, Showa University Fujigaoka Hospital, Yokohama, Japan.

Serum complement levels and hematological data were evaluated in five patients with immunoblastic lymphadenopathy (IBL) and four with IBL-like T cell lymphoma (IBL-T). Anemia with Hb values below 10.0 g/dl was seen in four patients. A direct Coombs test was positive in five patients and the bone marrow of two of these showed features of pure red cell aplasia. Seven patients were thrombocytopenic with platelet counts below 100 x 10(9)/l. Six of the seven patients had splenomegaly. Platelet-associated IgG was elevated in all three thrombocytopenic patients examined. Whole complement activity (CH50) was reduced in eight patients (89%) at presentation and subsequently normalized in five who were treated either with prednisolone (two patients with IBL) or with multidrug combination chemotherapy (three with IBL-T). One patient achieved complete remission and four partial remission. Remission was accompanied by normalization of hematological abnormalities and elevation of complement activity to the normal range in all cases. These results suggested that complement-mediated mechanisms are responsible, at least in part, for some of the hematological abnormalities observed in IBL and IBL-T and that hypocomplementemia is a common abnormality with significance as a laboratory marker for the disease activity.

UI MeSH Term Description Entries
D007119 Immunoblastic Lymphadenopathy A disorder characterized by proliferation of arborizing small vessels, prominent immunoblastic proliferations and amorphous acidophilic interstitial material. Clinical manifestations include fever, sweats, weight loss, generalized lymphadenopathy and frequently hepatosplenomegaly. Angioimmunoblastic Lymphadenopathy,Lymphadenopathy, Immunoblastic,Angioimmunoblastic Lymphadenopathies,Immunoblastic Lymphadenopathies,Lymphadenopathies, Angioimmunoblastic,Lymphadenopathies, Immunoblastic,Lymphadenopathy, Angioimmunoblastic
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D011239 Prednisolone A glucocorticoid with the general properties of the corticosteroids. It is the drug of choice for all conditions in which routine systemic corticosteroid therapy is indicated, except adrenal deficiency states. Di-Adreson-F,Predate,Predonine,Di Adreson F,DiAdresonF
D012010 Red-Cell Aplasia, Pure Suppression of erythropoiesis with little or no abnormality of leukocyte or platelet production. Aplasia Pure Red Cell,Erythrocyte Aplasia,Pure Red-Cell Aplasia,Aplasia, Erythrocyte,Aplasia, Pure Red-Cell,Aplasias, Erythrocyte,Erythrocyte Aplasias,Pure Red Cell Aplasia,Pure Red-Cell Aplasias,Red Cell Aplasia, Pure,Red-Cell Aplasias, Pure
D001853 Bone Marrow The soft tissue filling the cavities of bones. Bone marrow exists in two types, yellow and red. Yellow marrow is found in the large cavities of large bones and consists mostly of fat cells and a few primitive blood cells. Red marrow is a hematopoietic tissue and is the site of production of erythrocytes and granular leukocytes. Bone marrow is made up of a framework of connective tissue containing branching fibers with the frame being filled with marrow cells. Marrow,Red Marrow,Yellow Marrow,Marrow, Bone,Marrow, Red,Marrow, Yellow
D003165 Complement System Proteins Serum glycoproteins participating in the host defense mechanism of COMPLEMENT ACTIVATION that creates the COMPLEMENT MEMBRANE ATTACK COMPLEX. Included are glycoproteins in the various pathways of complement activation (CLASSICAL COMPLEMENT PATHWAY; ALTERNATIVE COMPLEMENT PATHWAY; and LECTIN COMPLEMENT PATHWAY). Complement Proteins,Complement,Complement Protein,Hemolytic Complement,Complement, Hemolytic,Protein, Complement,Proteins, Complement,Proteins, Complement System
D003176 Complement C3 A glycoprotein that is central in both the classical and the alternative pathway of COMPLEMENT ACTIVATION. C3 can be cleaved into COMPLEMENT C3A and COMPLEMENT C3B, spontaneously at low level or by C3 CONVERTASE at high level. The smaller fragment C3a is an ANAPHYLATOXIN and mediator of local inflammatory process. The larger fragment C3b binds with C3 convertase to form C5 convertase. C3 Complement,C3 Precursor,Complement 3,Complement C3 Precursor,Complement Component 3,Precursor-Complement 3,Pro-C3,Pro-Complement 3,C3 Precursor, Complement,C3, Complement,Complement, C3,Component 3, Complement,Precursor Complement 3,Precursor, C3,Precursor, Complement C3,Pro C3,Pro Complement 3
D003181 Complement C4 A glycoprotein that is important in the activation of CLASSICAL COMPLEMENT PATHWAY. C4 is cleaved by the activated COMPLEMENT C1S into COMPLEMENT C4A and COMPLEMENT C4B. C4 Complement,C4 Complement Component,Complement 4,Complement C4, Precursor,Complement Component 4,Pro-C4,Pro-complement 4,C4, Complement,Complement Component, C4,Complement, C4,Component 4, Complement,Component, C4 Complement,Pro C4,Pro complement 4
D003298 Coombs Test A test to detect non-agglutinating ANTIBODIES against ERYTHROCYTES by use of anti-antibodies (the Coombs' reagent.) The direct test is applied to freshly drawn blood to detect antibody bound to circulating red cells. The indirect test is applied to serum to detect the presence of antibodies that can bind to red blood cells. Anti-Human Globulin Consumption Test,Antiglobulin Consumption Test,Antiglobulin Test,Coombs' Test,Antihuman Globulin Consumption Test,Direct Antiglobulin Test,Direct Coombs Test,Indirect Antiglobulin Test,Indirect Coombs Test,Anti Human Globulin Consumption Test,Antiglobulin Consumption Tests,Antiglobulin Test, Direct,Antiglobulin Test, Indirect,Antiglobulin Tests,Antiglobulin Tests, Direct,Antiglobulin Tests, Indirect,Consumption Test, Antiglobulin,Consumption Tests, Antiglobulin,Coomb Test,Coomb's Test,Coombs Test, Direct,Coombs Test, Indirect,Direct Antiglobulin Tests,Indirect Antiglobulin Tests,Test, Antiglobulin,Test, Antiglobulin Consumption,Test, Coombs,Test, Coombs',Test, Direct Antiglobulin,Test, Direct Coombs,Test, Indirect Antiglobulin,Test, Indirect Coombs,Tests, Antiglobulin,Tests, Antiglobulin Consumption,Tests, Direct Antiglobulin,Tests, Indirect Antiglobulin

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