Everything you need to know about blood disorders
Hemophilia is a group of inherited blood disorders in which the blood does not clot properly. Hemophilia is the standard international spelling, also known as haemophilia in the UK, other translations include: hémophilie, hemofilie, hemofili, hemofilia, hämophilie, emofilia. We will use the standard international spelling for the purpose of this section. Hemophilia is an inherited bleeding disorder in which a […]
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Introduction: Hemophagocytic Lymphohistiocytosis (HLH) or Hemophagocytosis is a rare but potentially fatal disease of normal but overactive histiocytes and lymphocytes that commonly appears in infancy, although it has been seen in all age groups. Hemophagocytosis involves a defect in targeted killing and the inhibitory controls of natural killer and cytotoxic T cells, resulting in excessive cytokine production and accumulation of […]
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Hereditary Hemochromatosis (HH) is a genetic disorder that causes the body to absorb too much iron (Fe) from the diet. The excess iron is stored in the body’s tissues and organs, particularly the skin, heart, liver, pancreas, and joints. Because humans cannot increase the excretion of iron, excess iron can overload and eventually damage tissues and organs. For this reason, hereditary hemochromatosis […]
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Curing cancer requires eliminating all cancer cells. The major modalities of therapy are: Surgery (for local and local-regional disease): Surgery is the removal of the tumor and surrounding tissue during an operation. A doctor who specializes in treating cancer using surgery is called a surgical oncologist. Surgery is the oldest type of cancer therapy and remains an effective treatment for many types […]
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Hematologists are increasingly involved in the diagnosis and management of patients with venous and arterial thrombotic disorders. There have been major advances in recent years in our understanding of the central role of hypercoagulability in the pathogenesis of thrombosis. This has led to new approaches to the diagnosis of patients at risk for thrombosis and the development of more rational […]
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Stem cells are a class of undifferentiated cells that are able to differentiate into specialised cell types. Commonly, stem cells come from two main sources: Embryos formed during the blastocyst phase of embryological development (embryonic stem cells) and adult tissue (adult stem cells). Both types are generally characterised by their potency, or potential to differentiate into different cell types (such […]
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Qualitative platelet disorders are suggested by a prolonged bleeding time (abnormal platelet function screen) or clinical evidence of bleeding in the setting of a normal platelet count and coagulation studies. They are most commonly acquired, but can be inherited. Platelet function testing with light transmission aggregometry (LTA), the platelet function analyzer (PFA-100), or a template bleeding time may reveal abnormalities […]
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The hemostatic system consists of platelets, coagulation factors, and the endothelial cells lining the blood vessels. The platelets arise from the fragmentation of the cytoplasm of megakaryocytes in the bone marrow and circulate in blood as disc-shaped anucleate particles for 7-10 days. About one third are always transiently sequestered in the spleen. Platelets are eventually destroyed by apoptosis, a process independent […]
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First described in 1848, multiple myeloma (MM), also known as plasma cell myeloma, is characterized by a proliferation of malignant plasma cells and a subsequent overproduction of monoclonal paraprotein (M protein). There are several types of plasma cell neoplasms. These diseases are all associated with a monoclonal (or myeloma) protein (M protein). They include monoclonal gammopathy of undetermined significance (MGUS), […]
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Myelodysplastic Syndrome (MDS) is a group of diverse bone marrow disorders sometimes referred to as preleukemia, in which the bone marrow does not produce enough healthy blood cells. MDS affects hematopoiesis at the stem cell level, as indicated by cytogenetic abnormalities, molecular mutations, and morphologic and physiologic abnormalities in maturation and differentiation of one or more of the hematopoietic cell lines. […]
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