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62 Cards in this Set
- Front
- Back
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Heparin
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increases PTT, activates antithrombin III
affects intrinsic pathway |
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heparin antidote
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protamine sulfate
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warfarin
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increases PT
inhibits vitamin K and extrinsic pathway Goal INR 2-3 |
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warfarin antidote
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vitamin k
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INR goal w/ mechanical valves
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2.5 - 3.5
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VII deficiency
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elevated PT
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Hemophiila A
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FVIII deficiency
x-linked inheritance prolonged PTT normal PT, thrombin time, fibrinogen, bleeding time |
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Hemophilia B
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FIX deficiency
x-linked inheritance prolonged PTT normal PT, thrombin time, fibrinogen, bleeding time |
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Hemophilia diagnosis
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1. mixing study (patient plasma + normal plasma)
If correct: factor deficiency doesn't correct; clotting factor inhibitor 2. factor assay levels - mild: > 5% - moderate 1 - 3% - severe 1% or less |
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Hemophilia treatment
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transfusion of clotting factor (or cryoprecipitate) to at least 40% of normal concentration
DDAVP may help release extra VIII (also helps secrete von wiebrand factor) |
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von Willebrand's disease
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autosomal dominant
PT normal, PTT prolonged |
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von Willebrand's disease diagnosis
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ristocetin cofactor assay - measures capacity of vWF to agglutinate platelets)
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von Willebrand's disease Tx
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DDAVP - helps secrete vWF
AVOID aspirin or platelet inhibitors |
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Disseminated Intravascular Coagulation
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deposition of fibrin in small blood vessels, leads to thrombosis and end organ damage
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Disseminated Intravascular Coagulation Labs
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prolonged PT, PTT, thrombin and D-dimers
Acutely, platelet and clotting factors are decreased, chronically, they are normal |
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Thrombotic Thrombocytopenia Purpura
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fever
altered mental status Thrombocytopenia Microangiopathic anemia Renal failure |
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Thrombotic Thrombocytopenic Purpura Tx
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Steroids
Plasmapheresis |
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Idiopathic Thrombocytopenic Purpura
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commonly childhood hemorrhagic complications following virla illness
or women w/ child bearing age |
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Idiopathic Thrombocytopenic Purpura Dx
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diagnosis of exclusion
bone marrow biopsy would show megakaryocytes |
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Idiopathic Thrombocytopenic Purpura Tx
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most in childhood remit spontaneously
Main therapies: steroids, IVIG, splenectomy |
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Causes of microcytic anemia
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TICS
Thalassemia Iron deficiency Chronic disease Sideroblastic anemia |
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Iron deficiency labs
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low serum iron
high TIBC/transferrin low ferritin |
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Anemia of chronic disease labs
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low iron
low TIBC/transferrin high ferritin |
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B12 deficiency
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elevated methylmalonic acid and homocysteine
demyelinating disorders possible |
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Folate deficiency
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normal MMA, elevated homocysteine
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Polycythemia vera diagnosis
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All polycythemia's have elevated RBC's, WBC's and platelets
EPO is low in polycythemia vera, and normal in other polycythemias |
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Polycthemia Tx
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phlembotomy to reduce symptoms
address underlying cause |
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Polycythemia Vera Tx
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1. cryoreductive drugs (hydroxyurea / interferon)
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Prophalyxis to transfusion reactions
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acetominophen and diphenhydramine
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Porphyria
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Photodermatitis
neuropsychiatric complaints pink urine Seizures Colicky abdominal pains tachycardia, skin erythema and blisters areflexia |
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Porphyria Dx
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h&p
elevated blood urine, and stool porphyrins |
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Porphyria Tx
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high doses of glucose for mild attacks
IV hematin for severe attacks |
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Porphyria case
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college student drinks alcohol and barbiturates at a party, then has abdominal pain and brown urine next day
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Most common childhood malignancy
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ALL
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Acute leukemias
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anemia (fatigue, palor), thrombocytopenia (petechiae, purpura, bleeding), bone pains (ALL), splenomegaly
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ALL dx
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Bone marrow biopsy and aspiration
increased lymphoblasts - smaller, less cytoplasm inconspicuous nuclei myeloperoxidase - |
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AML dx
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bone marrow biopsy and aspiration
increased myeloblasts - larger, more cytoplasm, conspicuous nuclei, auer rods myeloperoxidase + |
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Preventing tumor lysis syndrome
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allopurinol - prevents hyperuricemia nd renal insufficiency
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Treatment for M3 AML
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Acute promyelocytic leukemia
Tx: all-trans-retinoic acid |
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CLL
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well differentiated B lymphocytes in older adults
Hx: fatigue, malaise, infection, lymphadenopathy, splenomegaly |
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CLL Dx
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flow cytometry: presence of CD5 on leukemic cells (normally only on T cells)
CD20 and CD21 Smudge cells on peripheral smere Abnormal function by leukemic cells leads to hypogammaglobulinemia |
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CLL Tx
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palliative treatment withheld until patients are symptomatic (recurrent infections, severe lymphadenopathy, splenomegaly, anemia, thrombocytopenia)
Tx: chemothreapy w/ akylating agents |
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CML
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BCR-ABL translocation
middle aged patients > 90% have Philadelphia chromosome t(9,22) |
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CML dx
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high WBC (100,000)
low leukocyte alkaline phosphatase high LDH, uric acid, b12 |
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CML tx
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Chronic (splenomegaly, LUQ pain): imatinib, stem cell transplant for young
- Imatinib (Gleevec) selective inhibitor of BCR-ABL tyrosine kinase Blast crisis (like acute leukemia): dastinib pulus hematopoietic cell transplant |
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Hairy Cell leukemia
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pancytopenia, bone marrow infiltration, splenomegaly
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Hairy Cell Leukemia Dx
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tartrate-resistant acid phosphatase (TRAP) staining
CBC: Hairy cells - mononuclear cells w/ abndant pale cytoplasm and cytoplasmic projections |
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Hairy cell leukemia tx
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nucleoside analogs - induce remission
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Lymphomas
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malignant transformation of lymphoid cells residing primarily in lymphoid tissues, especially lymph nodes
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Non-Hodgkin's Lymphoma
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Most are B-cell origin
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Non-Hodgkin's Lymphoma Dx
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1. excisional lymph node biopsy
2. disease staging based on number of nodes and whether the disease crosses the diaphragm |
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Non-Hodgkin's Lymphoma Tx
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CHOP
Cyclophosphamide, Adriamycin, Oncovin (vincristine), prednisone 1. low-grade indolent NHL: palliative approach 2. high grade: CHOP |
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Hodgkins lymphoma
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cervical adenopathy or mediastinal mass
B symptoms, pruritus, hepatosplenomegaly, Pel-Ebstein fevers (weeks of fever alternating w/ weeks of afebrile) Alcohol induced pain Bimodal Age distribtion - third decade (nodular sclerosing) - 60 (lymphocyte depleted) |
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Hodgkin's lymphoma
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excisional lymph node biopsy: reed sternberg cells, owl eye appearance w/ eosinophilic nuclei
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Hodgkin's lymphoma tx
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1. radiation directed towards lymph nodes
Chemo: ABVD and MOPP Adriamycin, bleomycin, vinblastine, dacarbazine Mechlorethamine, Oncovin (vincristine), procarbazine, prednisone |
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Multiple myeloma
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excess production of monoclonal immunoglobulins or immunogloblin fragments
CRAB hypercalcemia renal impairment Anema Bone pains/lytic lesions/fractures Think bone pain at rest Prone to infections and elevated monoclonal M proteins |
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Multiple myeloma dx
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> 10% plasma cells in bone marrow, M protein in serum or urine, evidence of lytic bone lesions
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Multiple myeloma tx
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Chemotherapy
Melphalan and prednisone |
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Waldenstrom's Macroglobulinemia
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Elevated IgM leading to hyperviscosity, coagulation abnormalities, cryoglobulinemia, cold agglutinin disease, amyloidosis
Chronic indolent disease of elderly |
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Waldenstrom's Macroglobulinemia Dx
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Elevated ESR, uric acid, LDH, alkaline phosphatase
Bone marrow aspirate shows plasma cells, PAS + |
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Waldenstrom's Macrogloblinemia
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1. remove excess IgM w/ plasmapharesis
2. Treat underlying lymphoma w/ chemotherapy |
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Amyloid AL
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multiple myeloma and Waldenstrome's macroglobulinemia
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