Medical Blog

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 disease, are the most common causes. Up to 50% of patients with severe liver disease secondary to cirrhosis, hepatoma, or hepatitis exhibit bleeding complications. Read more…

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).
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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 actions are catalyzed by the interaction between antithrombin and vessel wall-associated glycosaminoglycans. Recent studies have also shown that antithrombin has anti-inflammatory actions that are independent of its effect on coagulation.7,8,9  Read more…

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 not widely appreciated. Read more…

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).
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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 of patients with follicular lymphomas is approximately 8-10 years, and many patients may not require treatment for prolonged periods of time.
Pathophysiology Read more…

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 myeloma can range from asymptomatic to very symptomatic with complications requiring emergent treatment. Systemic ailments include bleeding, infection and renal failure, and local catastrophes, including pathologic fractures and spinal cord compression. Although patients benefit from treatment (ie, longer life, less pain, fewer complications), currently no cure exists. Recent advances in therapy have helped to lessen the occurrence and severity of adverse effects of multiple myeloma. Read more…

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. Read more…

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 times the total WBC count. Read more…

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). Read more…