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OPT Hematologic
| Term | Definition |
|---|---|
| lymphoid hyperplasia | enlargement of lymph nodes in response to infection; can be acute or chronic |
| hemophilia | variety of bleeding disorders due to a hereditary deficiency of coagulation factors |
| hemophilia A (classic hemophilia) | most common type of hemophilia; decreased factor VIII |
| hemophilia B (Christmas disease) | characterized by low levels of factor IX, very similar to hemophilia A |
| von Willebrand disease | deficiency of vWF that aids in platelet adhesion and stabilization of factor VIII; causes prolonged bleeding and coagulation |
| plasminogen deficiency | rare autosomal recessive condition of low levels of plasminogen, a precursor to plasmin that breaks down fibrin; leads to irregular plaques and nodules especially in the eyes |
| ligneous | type of thick, firm plaque on conjunctiva that accumulate due to plasminogen deficiency |
| anemia | decrease in volume of RBCs (hematocrit) or in hemoglobin concentration, resulting in decreased oxygen delivery |
| sickle cell anemia | codominant disorder of hemoglobin synthesis, confers resistance to malaria; can see hair-on-end skull films |
| sickle cell trait | only 1 hemoglobin allele is affected so 40-50% of hemoglobin is abnormal; symptoms only appear at low oxygen |
| sickle cell disease | both hemoglobin alleles are affected, so RBCs bucks and can block capillaries; results in chronic hemolytic anemia |
| sickle cell crisis | arises from hypoxia, infection, hypothermia or dehydration; severe pain from ischemia and infarction |
| thalassemia | hereditary disorders of hemoglobin synthesis, deficiency of alpha or beta hemoglobin chains |
| beta-thalassemia | insufficient beta chains produces so excess alpha chains |
| thalassemia minor | one defective gene of insufficient beta-chains; no significant alterations or symptoms |
| thalassemia major (Cooley anemia) | 2 defective genes of insufficient beta-chains; Chipmunk facies and hair-on-end skull films |
| alpha-thalassemia | deficient alpha-chains with excess beta-chains; 4 genes involved |
| alpha-thalassemia trait | 2/4 genes for alpha-chain deleted, insignificant levels of anemia and microcytosis |
| hemoglobin H (HbH) disease | 3/4 genes for alpha-chain deleted; significant hemolytic anemia and splenomegaly |
| hydrops fetalis | 4/4 genes for alpha-chain deleted; generalized edema and fatal within a few hours of birth |
| aplastic anemia (pancytopenia) | secondary to failure of marrow stem cells leading to deficiencies in all blood cells |
| neutropenia | circulating neutrophils below 1500/mm^3, variety of different causes presenting in mouth as oral ulcers and enhanced periodontitis |
| agranulocytosis | absence of granulocytic cells, especially neutrophils; most secondary to medication and presents as ANUG and oral ulcers |
| cyclic neutropenia | idiopathic disorder of periodic reductions in neutrophils from mutation of {neutrophil elastase gene} that disrupts neutrophil maturation and triggers premature apoptosis |
| neutrophil elastase gene | mutation disrupts neutrophil maturation and triggers premature apoptosis and leads to cyclic neutropenia |
| thrombocytopenia | decreased circulating platelets; can be due to decreased marrow production or increased platelet destruction {TTP, ITP} |
| thrombotic thrombocytopenic purpura (TTP) | deficiency in metalloprotease that cleaves vWF; forms numerous thrombi in small vesicles |
| immune thrombocytopenic purpura (ITP) | secondary to development of anti platelet antibodies following viral infection and leads to destruction of the spleen |
| polycythemia vera | rare uncontrolled proliferative disorder of marrow resulting in 2-3x more RBCs, usually in older adults |
| erythromelalgia | burning, erythema, and warmth of hands and feet with high temperature or exercise |
| leukemia | group of malignancies that airs from marrow stem cells that spill into peripheral blood; can be acute/chronic and lymphoblastic/myeloid |
| acute lymphoblastic leukemia | leukemia almost always in childhood, 90% cure rate |
| chronic lymphoblastic leukemia | leukemia in elderly adults, considered incurable |
| chronic myeloid leukemia | leukemia with peak frequency in 3rd and 4th decade; good prognosis of 80% |
| acute myeloid leukemia | leukemia that affects a wide range of ages; 5 year survival rate of 40% |
| myelophthisic anemia | severe anemia, thrombocytopenia and leukopenia seen in association with leukemia |
| myeloid sarcoma | soft tissue involvement of leukemia associated with myelomonocytic variants |
| chloroma | nickname for mass of leukemia cells that exhibit greenish coloration |
| Langerhans cell histiocytosis | proliferation of histiocyte-like Langerhans cells mostly under 15 year olds; dental manifestations of teeth floating in air |
| Birbeck granule | rod-shaped structure in cytoplasm of Langerhans cell |
| eosinophilic granuloma | clinical presentation of Langerhans cell histiocytosis with solitary or multiple bone lesions |
| Hand-Schuller-Christian disease | Langerhans cell histocytois presentation of chronic disseminated disease |
| Letterer-Siwe disease | Langerhans cell histiocytosis presentation of acute disseminated disease |
| Hodgkin lymphoma | lymphoreticular malignancy with large, unique {Reed-Sterberg cells}; originates as B-lymphocytes and is associated with EBV |
| Non-Hodgkin lymphoma | malignancies of lymphocytes originating in nodes and from B-cells; associated with H. pylori, EBV and HHV-8 |
| low-grade hymphomas | non-Hodgkin lymphoma that does not respond well to therapy, not usually treated |
| intermediate lymphomas | non-Hodgkin lymphoma with 50-70% survival |
| high-grade lymphomas | non-Hodgkin lymphoma with 40% 5 year survival rate |
| mycosis fungoides | malignancy of CD-4 T-cells with propensity to involve epidermis: eczematous > plaque > tumor |
| Sezary syndrome | aggressive dermatopathic T-cell leukemia with wide involvement of organs |
| Burkitt lymphoma | malignancy of undifferentiated B-cells associated with EBV and has a predilection for the jaws {endemic / African Burkitt lymphoma} |
| sporadic / American Burkitt lymphoma | similar malignancy detected as abdominal mass, type of Burkitt lymphoma |
| immunodeficiency-associated Burkitt lymphoma | HIV-related lymphomas with features of Burkitt lymphoma |
| starry-sky | cellular infiltrate of dense sheets of small lymphocytes mixed with benign macrophages |
| extranodal NK/T-cell lymphoma, nasal type | aggressive destruction of midline structures of palate and nose; unusual pattern of T-cell lymphoma, typically in adults |
| multiple myeloma | monoclonal malignancy of plasma cells with multi centric origin in bone; symptom of bone pain |
| plasmacytomas | similar to multiple myeloma; unifocal bone or soft tissue proliferations |
| monoclonal gammopathy | massive overproduction of Ig-generated by the neoplastic clone of plasma cells |
| Bence Jones proteins | excess light chains detected in urine in 30-50% of multiple myeloma cases |
Created by:
aharnold
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