Everything you need to know about blood disorders
Acute Myeloid Leukemia (AML) is a malignant proliferation of myeloblasts in the blood and bone marrow. The hematopoietic precursors are arrested in an early stage of development. Most AML subtypes are distinguished from other related blood disorders by the presence of more than 20% blasts in the bone marrow. The disease is commonest in the middle-aged and elderly. Clinical Features: Although the onset […]
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Acute Lymphoblastic Leukemia (ALL) is a malignant proliferation of lymphoblasts in the blood and bone marrow. The disease is usually rapidly progressive if untreated. The incidence of ALL peaks in childhood and then remains relatively constant at lower levels throughout adult life. Two-thirds of all ALL cases occur in children, with a peak incidence at age 2 to 5 years; […]
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Mycosis Fungoides (MF) and Sezary Cell Leukemia (SCL) are related T-cell cutaneous lymphoma/leukemia. Clinical Features: Mycosis Fungoides is an uncommon chronic T-cell lymphoma primarily affecting the skin and occasionally the internal organs. The disease is most common in middle-aged men. There are eczematous lesions, plaques, tumors, and ulcers of the skin without overt blood involvement. In the late stages, the […]
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Primary Myelofibrosis (PMF) is a chronic myeloproliferative disorder characterized by bone marrow fibrosis, splenomegaly, and anemia with nucleated and teardrop-shaped RBCs. The spleen is often very large. Myelofibrosis may be primary or secondary to a number of hematologic, malignant, and nonmalignant conditions e.g. PRV, ET, hairy cell leukemia, cancer with bone marrow metastases, osteomyelitis and TB. PMF is more common than […]
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Essential thrombocythemia (ET) is a myeloproliferative neoplasm (MPN) characterized by megakaryocyte hyperplasia and thrombocytosis due to a clonal abnormality of a multipotent hematopoietic stem cell. From the genetic perspective, ET patients harbour mutations in JAK2 (50–60%), CALR (15–30%) and MPL (1–5%) genes. Platelet survival is normal in ET. Approximately one-third of patients with ET is asymptomatic at diagnosis. ET usually occurs […]
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Polycythemia Rubra Vera (PRV) or Primary Polycythemia Vera is a chronic myeloproliferative disorder characterized by an increase in RBC mass, which often manifests as an increased hematocrit (Hct). There is a malignant transformation of the multipotential stem cell with excess production of red cells. PRV involves increased production of all cell lines, including RBCs, WBCs, and platelets. Polycythaemia Rubra Vera […]
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Chronic myeloid leukemia (CML), also known as chronic myelogenous leukemia, is a myeloproliferative disorder characterized by increased proliferation of the granulocytic cell line without the loss of their capacity to differentiate. Aetiology: In chronic myeloid leukemia (CML) there is a malignant transformation of the multipotential hematopoietic stem cells (pluripotent stem cells). There are excessive cells in the granulocytic series, primarily in […]
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Langerhan’s Cell Histiocytosis (LCH), also called Histiocytosis X, is a group of diseases ranging from the relatively benign unifocal eosinophilic granuloma, to the multifocal variant, to the very malignant diffuse histiocytic infiltration previously known as the Letterer-Siwe syndrome which is frequently fatal. This disorder is characterized by an abnormal increase in certain immune cells called histiocyte cells. Histiocyte cells are […]
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Gaucher’s Disease is an autosomal recessive condition most common in Jews. There is β-glucocerebrosidase deficiency causing deposition of glucocerebroside and related compounds, leading to hepatosplenomegaly, CNS changes and bony infiltration. Glucocerebrosidase normally hydrolyzes glucocerebroside to glucose and ceramide. Genetic defects of the enzyme cause glucocerebroside accumulation in tissue macrophages through phagocytosis, forming Gaucher cells (with onion skin appearance). Accumulation of […]
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Sea Blue Histiocytes are large lipid-containing histiocytes in the bone marrow which stain sea blue in color. They may be found in a rare autosomal dominant condition characterized by neurological impairment and splenomegaly. Sea-blue–colored histiocytes have been described in the setting of high rates of intramedullary cell death due to lipid storage diseases (e.g. Niemann-Pick disease), myelodysplastic syndromes (MDS), lymphomas, […]
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