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Category: Hematology Articles

Dysfibrinogenemia ?>

Dysfibrinogenemia

Dysfibrinogenemia Introduction Background Congenital dysfibrinogenemia is a term used to describe a relatively rare condition wherein an inherited abnormality in the fibrin molecule results in defective fibrin clot formation. The complications associated with abnormal clot formation range from asymptomatic to life threatening. Fortunately, 40% of patients with congenital dysfibrinogenemia are asymptomatic; however, 50% of patients have a bleeding disorder and the remaining 10% have a thrombotic disorder or combined thrombotic and bleeding tendencies. Acquired dysfibrinogenemias, often called dysfibrinogenemia of liver…

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Disseminated Intravascular Coagulation ?>

Disseminated Intravascular Coagulation

Disseminated Intravascular Coagulation Introduction Background Disseminated intravascular coagulation (DIC) is not a specific diagnosis, and its presence always indicates another underlying disease. There are many diseases that may lead to the occurrence of disseminated intravascular coagulation (DIC) (see Causes).

Antithrombin Deficiency ?>

Antithrombin Deficiency

Antithrombin Deficiency Introduction Background Antithrombin III (ATIII) is currently referred to as antithrombin (AT). Antithrombin (AT) is a 58-kDa molecule belonging to the serine protease inhibitor (serpin) superfamily that plays a central role as an anticoagulant in mammalian circulation systems.1 In fact it is present in a wide variety of organisms ranging from thermophilic bacteria2 to mammals. In addition to its effect as an antagonist of thrombin, it also inhibits other proteases of the coagulation cascade3,4,5,6 (see Image 1). These…

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Alpha2-Plasmin Inhibitor Deficiency ?>

Alpha2-Plasmin Inhibitor Deficiency

Alpha2-Plasmin Inhibitor Deficiency Introduction Background Platelet disorders and inherited or acquired deficiencies of hemostatic factors (eg, factor VIII, factor IX, or von Willebrand factor [vWF]) lead to excessive bleeding, as is widely recognized. Widespread experience with the use of thrombolytic agents in acute myocardial infarction currently indicates that excess plasmin, generated by thrombolytic drugs, increases bleeding risk. However, the fact that a deficiency of alpha2-plasmin inhibitor (alpha 2-PI, a2-PI), a physiologic inhibitor of fibrinolysis, can lead to excessive bleeding is…

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Lymphoma, Malignant Small Noncleaved ?>

Lymphoma, Malignant Small Noncleaved

Lymphoma, Malignant Small Noncleaved Introduction Background Small noncleaved cell (SNCC) lymphomas are high-grade B-cell lymphoma. It is a historical term used to describe Burkitt lymphoma (BL). The World Health Organization Classification of lymphoid neoplasms identifies Burkitt lymphoma/leukemia as a highly aggressive mature B-cell neoplasm. It is further subdivided into endemic, sporadic, and immunodeficiency-associated variants. Some patients can also present with leukemia (L3 ALL).

Lymphoma, Follicular ?>

Lymphoma, Follicular

Lymphoma, Follicular Introduction Background Non-Hodgkin lymphoma is a heterogeneous group of malignancies of lymphocyte origin that usually arise or are present in lymphoid tissues, such as lymph nodes, spleen, and bone marrow. Nevertheless, lymphomas can arise in any organ and usually are referred to as primary extranodal lymphomas. Microscopically, follicular lymphomas exhibit a follicular or nodular pattern of growth reminiscent of germinal centers. Despite the fact that most follicular lymphomas are advanced at the time of diagnosis, the median survival…

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Multiple Myeloma ?>

Multiple Myeloma

Multiple Myeloma Introduction Background Multiple myeloma is a debilitating malignancy that is part of a spectrum of diseases ranging from monoclonal gammopathy of unknown significance (MGUS) to plasma cell leukemia. First described in 1848, multiple myeloma is a disease characterized by a proliferation of malignant plasma cells and a subsequent overabundance of monoclonal paraprotein. An intriguing feature of multiple myeloma is that the antibody-forming cells (ie, plasma cells) are malignant and, therefore, may cause unusual manifestations. The presentation of multiple…

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Splenomegaly ?>

Splenomegaly

Splenomegaly Introduction Background Splenic anatomy and function1 The spleen is a functionally diverse organ with active roles in immunosurveillance and hematopoiesis. It lies within the left upper quadrant of the peritoneal cavity and abuts ribs 9-12, the stomach, the left kidney, the splenic flexure of the colon, and the tail of the pancreas. A normal spleen weighs 150 g and is approximately 11 cm in craniocaudal length.

Neutropenia ?>

Neutropenia

Neutropenia Introduction Background Neutropenia is a decrease in circulating neutrophils in the peripheral blood.1 The absolute neutrophil count (ANC) number defines neutropenia. An abnormal ANC value contains fewer than 1500 cells per mm3. Blacks may have a lower but normal ANC value of 1000 cells per mm3, with a normal total white blood cell (WBC) count. The ANC is calculated by multiplying the percentage of bands and neutrophils (segmented neutrophils or granulocytes) on a complete blood cell (CBC) count differential…

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Eosinophilia ?>

Eosinophilia

Eosinophilia Introduction Background In this article, the term eosinophilia is defined as an increase in peripheral blood eosinophilic leukocytes to more than 600 cells per microliter of blood. Emphasis is placed on the number of eosinophils circulating in the peripheral blood, although an increase in eosinophils can be observed in other body fluids (eg, cerebrospinal fluid [CSF], urine) and many body tissues (eg, skin, lung, heart, liver, intestine, bladder, bone marrow, muscle, nerve).