Hematology/Oncology Flashcards
Prolonged prothrombin time
Defect in extrinsic coagulation system, factor VII
Osler-Weber-Rendu Syndrome
Hereditary hemorrhagic telangiectasia. Autosomal dominant. Presence of telangiectasias in the skin as well as mucous membranes of the lips, oropharynx, respiratory tract, GI tract, and urinary tract. Rupture may cause epistaxis, GI bleeding, or hematuria
What drives angiogenesis?
Vascular endothelial growth factor (VEGF) and Fibroblast growth factor (FGF)
What is HER2 positivity associated with in breast cancer?
Poorly differentiated, rapidly growing tumors
Kawasaki diagnosis criteria
Fever for greater than or equal to 5 days plus 4 of the following
- Bilateral non-exudative conjunctival injection
- Cervical lymphadenopathy
- Mucositis: Erythema of the palatine mucosa, fissured erythematous lips, “strawberry tongue”
- Extremity changes: Edema of the hands and feet, erythema of palms and soles, desquamation of the fingertips
- Rash: polymorphous erythematous rash on the extremities that spreads centripetally to the trunk
What is a serious complication of Kawasaki disease?
Coronary artery inflammation leading to the development of coronary artery aneurysms
t(15;17)
Acute promyelocytic leukemia (APL)
Warfarin induced skin necrosis can occur in individuals with what deficiency?
Underlying protein C or S deficiency
How do renal cortical cells respond to hypoxia?
Synthesize and release erythropoietin
When should inherited causes of hypercoagulability be considered?
In patients younger than 5 who present with thrombosis and no obvious explanation for an acquired prothrombotic state
Describe mechanism of factor V Leiden mutation
Causes factor Va resistance to inactivation by activated protein C
IgE-independent mast cell activation common symptoms
Diffuse itching and pain, bronchospasm, and localized swelling (urticaria)
What is the most common trigger of DIC in pregnancy?
Release of tissue factor (thromboplastin) from an injured placenta into the maternal circulation
Which arteries are typically spared in polyarteritis nodosa?
Pulmonary
What causes pancytopenia seen with lupus erythematosus?
Formation of autoantibodies against blood cells (type II hypersensitivity)
Pure red cell aplasia is associated with?
Thymoma, lymphocytic leukemias, parvovirus B19
Actions of active/inactive Rb protein
Active (hypophosphorylated) RB protein prevents damaged cells from preceding past G1 to S checkpoint, while inactive (hyperphosphorylated) Rb protein allows the damaged cells to enter mitosis.
What does binding of programmed death receptor 1 to its ligand do?
Down regulates the immune response against tumor cells by inhibiting cytotoxic T cells. Many types of cancers increase expression of PD-L1
Triad of eczema, thrombocytopenia, and combined B and T lymphocyte deficiency. Repeated infections by encapsulated organisms. Presents at 6-12 months of age.
The Wiskott-Aldrich syndrome
Li-Fraumeni Syndrome
Aut. dominant mutation in TP53. Leukemia, sarcomas, tumors of the breast, brain, and adrenal cortex are most common
Functions of vWF
Promoter of platelet adhesion at sites of vascular injury by binding platelet glycoproteins ti subendothelial collagen on injured blood vessels. Also acts as a protective carrier protein for circulating factor VIII
Anticoagulant reversal of heparin
Protamine sulfate - binds to heparin causing chemical inactivation
Treatment for PNH
Eculizumab - inhibits terminal complement formation
Mutation in sickle cell anemia
Single amino acid replacement in beta chain (substitution of glutamic acid with valine)
