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OPT Hematologic

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