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USMLE

HEME

TermDefinition
Tumor cell lysis Release of K+ hyperkalemia release of PO 3– hyperphosphatemia hypocalcemia due to Ca sequestration by PO43–. - Calcium phosphate crystals - nucleic acid breakdown hyperuricemia acute kidney injury RX; aggressive hydration, allopurinol, rasburicase.
polycythemia relative - DECREASE plasma VOLUMES---> INCREASE EPO - RBC low vera - increase RBC - decrease EPO - plasma volume > RX; phlebotomy, hydroxyurea, ruxolitinib (JAK1/2 inhibitor) inappropriate absolute - INCREASE EPO -Exogenous EPO: athlete abuse (“blood doping”). Inappropriate EPO secretion: malignancy - RBC > appropriate absolute - DECREASE O2 SAT - INCREASE EPO - RBC >
hyperuricemia polycythemia - treat with ruxolitinib (JAK1/2 inhibitor). tumor lysis syndrome
wide spread body itching obstructive jaundice - bile salt polycythemia vera - histamine
generalized non tender lymphadenopathy CLL pt > 60 smudge cells abnormal anemia lymphoblast < 30% blasts anemia thrombocytopenia
protein C and S anticoagulation inhibit factor v inhibit factor VIII
Heparin glucose amino glycan - mucopolysaccharide activates antithrombin -Antithrombin inhibits thrombin (factor IIa) ,VIIa, IXa, Xa, XIa, XIIa. inhibits factor Xa inhibits thrombin
birth control pill estrogen inhibits antithrombin 3 increases fibrinogen
prostacylin PG1 vasodilator - prevents aggregation of platelets anticoagulation - aspirin inhibits it
platelets, megakaryocytes vWF - in alpha granules vWF - also in wp bodies adhesion
ADP in dense granules of platelets - released after binding of platelets to vWf via Gp1b receptor helps platelets adhere to endothelium -binding to P2Y12 receptor induces GpIIb/IIIa expression at platelet surface -Fibrinogen binds GpIIb/IIIa receptors and links platelets Clopidogrel, prasugrel, ticlopidine - inhibit ADP-induced expression of GpIIb/IIIa - irreversibly blocking P2Y12 receptor.
TXA2 vasoconstriction - in prinzemetal agina (released by platelets) ↓ blood flow ↑ platelet aggregation decreases bleeding time dipyridamole
platelet disorder petechia increased bleeding time ecchymosis epistaxis easy superficial bleeding - Bernard-Soulier syndrome - Glanzmann - thrombastheniaImmune thrombocytopenia -Thrombotic thrombocytopenic purpura and hemolytic-uremic syndrome
Paroxysmal nocturnal hemoglobinuria defect in the PIGA gene prevents formation of glycosylphosphatidylinositol (GPI) - anchors for complement inhibitors -decay-acclerating factor (DAF/CD55) -membrane inhibitor of reactive lysis (MIRL/CD59) - complement-mediated intravascular hemolysis -haptoglobin, - dark urine
Early complement deficiencies (C1-C4) risk of severe, recurrent pyogenic sinus . respiratory tract infections. increased risk of SLE
Neutrophils Specific granules: - leukocyte alkaline - phosphatase (LAP), - collagenase, - lysozyme, lactoferrin. Azurophilic granules (lysosomes); - proteinases, - acid phosphatase, - myeloperoxidase, - β-glucuronidase left shift with band cells ; - (immature neutrophils) - states of myeloid proliferation , bacterial infections, CML
Mast cells Mediate local tissue allergic reactions. Contain basophilic granule Activated by tissue trauma, C3a and C5a -BIND the Fc portion of IgE to membrane. IgE cross-linking by antigen -->(IgE receptor aggregation) --> degranulation release of histamine, - heparin, tryptase, - eosinophil chemotactic factors. Vancomycin, opioids, and radiocontrast dye -- elicit IgE-independent mast cell degranulation.
Immune thrombocytopenia Anti-GpIIb/IIIa antibodies --> Destruction of platelets in spleen. -->splenic macrophages phagocytose platelets. idiopathic or 2° to autoimmune disorders (eg, SLE), - viral illness (eg, HIV, HCV), - malignancy (eg, CLL), - drug reactions. > megakaryocytes on bone marrow biopsy < platelet count > bleeding time RX: IVIG, rituximab, - TPO receptor agonists (eltrombopag, romiplostim), - splenectomy for refractory ITP.
Thrombotic thrombocytopenic purpura inhibition or deficiency of ADAMTS13 ( vWF metalloprotease) -<degradation of vWF multimers --> large vWF multimers --> platelet adhesion and aggregation (microthrombi formation) triad of ; thrombocytopenia, microangiopathic hemolytic anemia, acute kidney injury. (triad + fever + neurologic symptoms) platelet count, hemolytic anemia (eg, schistocytes, LDH). Normal PT/ PTT helps distinguish HUS and TTP (coagulation pathway is not activated) from DIC RX: plasmapheresis, steroids, rituximab.
Antithrombin deficiency inherited defect no direct effect on the PT, PTT, or thrombin time . diminishes the increase in PTT following heparin administration can acquired: renal failure/nephrotic syndrome --> increase antithrombin loss in urine ---> decrease inhibition of factors IIa and Xa.
Prothrombin gene mutation Mutation in 3′ untranslated region increase production of prothrombin increase plasma levels and venous clots.
Factor V Leiden mutant factor V (guanine adenine DNA point mutation Arg506Gln mutation near the cleavage site) is resistant to degradation by activated protein C. Complications include DVT, cerebral vein thrombosis, recurrent pregnancy loss
Mycosis fungoides/ Sézary syndrome skin patches and plaques (cutaneous T-cell lymphoma), - atypical CD4+ cells with “cerebriform” nuclei - intraepidermal neoplastic cell aggregates (Pautrier microabscess). May progress to Sézary syndrome (T-cell leukemia).
Acute lymphoblastic leukemia/lymphoma ALL - CHILDREN 0-14 less common in adults (worse prognosis) -Associated with Down syndrome. mediastinal mass (presenting as SVC-like syndrome). - Tcell - lymphoblasts on blood smear TdT+ (marker of pre-T and pre-B cells) -CD10+ (marker of pre-B cells). - Most responsive to therapy. - May spread to CNS and testes. - t(12;21) better prognosis
Chronic lymphocytic leukemia/small lymphocytic lymphoma CD20+, CD23+, CD5+ B-cell neoplasm. - Often asymptomatic, - progresses slowly; - smudge cells in peripheral blood smear; autoimmune hemolytic anemia. richter transformation—CLL/SLL transformation into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma (DLBCL)
Hairy cell leukemia - Mature B-cell tumor. - Cells have filamentous, hair-like projections (fuzzy appearing on LM ). Peripheral lymphadenopathy is uncommon. - marrow fibrosis - dry tap on aspiration. - usually present with massive splenomegaly and pancytopenia. - Stains TRAP (tartrate-resistant acid phosphatase) ⊕ - BRAF mutations. RX; cladribine, pentostatin.
Acute myelogenous leukemia Median onset 65 years. - circulating myeloblasts on peripheral smear. Auer rods ; myeloperoxidase ⊕ cytoplasmic inclusions - seen mostly in APL -APL: t(15;17) - DIC is a common presentation Risk factors: prior exposure to alkylating chemotherapy, - radiation, - myeloproliferative disorders, - Down syndrome. all-trans retinoic acid (vitamin A) and arsenic - induce differentiation of promyelocytes
Chronic myelogenous leukemia 45–85 years -philadelphia chromosome (t[9;22], BCR-ABL - myeloid stem cell proliferation. --> dysregulated production of mature and maturing granulocyte --> splenomegaly. - May accelerate and transform to AML or ALL (“blast crisis”). - Very low leukocyte alkaline phosphatase (LAP) as a result of low activity in malignant neutrophil RX: bcr-abl tyrosine kinase inhibitors (eg, imatinib, dasatinib)
myelodysplastic - Stem cell disorders - ineffective hematopoiesis defects in cell maturation of nonlymphoid lineages - by de novo mutations -environmental exposure (eg, radiation, benzene, chemotherapy). Risk of transformation to AML.
Adult T-cell lymphoma HTLV (associated with IV drug abuse) cutaneous lesions; - Lytic bone lesions, -hypercalcemia common in Japan (T-cell in Tokyo), West Africa, and the Caribbean.
Hemophagocytic Lymphohistocytosis macrophages and T cells involved - systemic over ativation fever, splenomegaly, pancytopenia >>>Ferritin macrophages seen phagocytosing marrow cells inherited or 2° to strong immunologic activation (eg, after EBV infection, malignancy
ADP receptor inhbitors Clopidogrel, prasugrel, ticagrelor (reversible), ticlopidine. irreversibly blocking ADP (P2Y12) receptor. Inhibit platelet aggregation -Acute coronary syndrome; coronary stenting. - decrease incidence or recurrence of thrombotic stroke. Neutropenia (ticlopidine). TTP may be seen
Cilostazol, dipyridamole. antiplatelet phosphodiesterase inhibitors cAMP in platelet --> inhibition of platelet aggregation; - vasodilators. prevention of stroke or TIAs (combined with aspirin) AVR: Nausea, headache, facial ushing, hypotension, abdominal pain.
Abciximab, eptifibatide, tirofi ban. glycoprotein IIa/IIIb inhibitors - Unstable angina, - percutaneous coronary intervention. Bleeding, thrombocytopenia.
Hydroxyurea: inhibits ribonucleotide reductase RX: sickle cell, polycythemia vera
Drugs work on S phase and G2 phase of cell cycle Etoposide/teniposide: inhibits topoisomerase II targets DNA
Drugs independent of cell cycle that also target DNA alkylating agents, busulfan Cyclophosphamide Ifosfamide Nitrosoureas (eg, carmustine) platinum agents: cross-link DNA
Doxorubicin Generate free radicals. Intercalate in DNA breaks in DNA replication. Interferes with topoisomerase II enzyme RX; Solid tumors, leukemias, lymphomas. myelosuppression, alopecia. Dexrazoxane (iron chelating agent) used to prevent cardiotoxicity.
Cladribine inhibition of DNA polymerase, DNA strand breaks). RX: Hairy cell leukemia Myelosuppression, nephrotoxicity, and neurotoxicity.
Cytarabine (arabinofuranosyl cytidine) Pyrimidine analog DNA chain termination. At higher concentrations, inhibits DNA polymerase. Leukemias (AML), lymphomas. Myelosuppression with megaloblastic anemia. CYTarabine causes panCYTopenia.
Cyclophosphamide, ifosfamide Cross-link DNA at guanine. Require bioactivation by liver - rheumatic disease (eg, SLE, granulomatosis with polyangiitis). - solid tumors, leukemia, lymphomas AVR; Fanconi syndrome (ifosfamide) AVR; Myelosuppression; SIADH
Alkylating agents that Require bioactivation. all alkylating agents crosslinks DNA - cell cycle independent - Cyclophosphamide, ifosfamide - Nitrosoureas (eg, carmustine, lomustine) -Procarbazine Nitrosoureas -Cross blood-brain barrier -Brain tumors (including glioblastoma multiforme). Procarbazine -Hodgkin lymphoma, brain tumors. AVR: Bone marrow suppression, pulmonary toxicity, leukemia, disulFram-like reaction
Fetal erythropoiesis Yolk sac (3–8 weeks) ƒ Liver (6 weeks–birth) ƒ Spleen (10–28 weeks) ƒ Bone marrow (18 weeks to adult)
RBC type O - no antigen on surface - anti A and anti B antibodies in plasma - IgM, IgG - Universal donor of RBCs; universal recipient of plasma AB - antigens A, B - no antibodies - Universal recipient of RBCs; universal donor of plasma A - antigen A - antibodies B in plasma (IgM) B - antigen B - antibodies A in plasma.
Rh - MOTHER Rh + FETUS no antigen on RBC surface - Anti D IgG in plasma Anti D IgG in plasma crosses the placenta. erythroblastosis fetalis. Rh hemolytic disease of the newborn Jaundice shortly after birth, - kernicterus, - hydrops fetalis.
Basophilic stippling Aggregation of ribosomal precipitates. Sideroblastic anemias (eg, leadpoisoning, myelodysplastic syndromes), thalassemias. peripheral smear, vs ringed sideroblasts seen in bone marrow.
Heinz bodies G6PD deficiency. G6PD deficiency --> can not reduce gluthatione Oxidative stress Hb denatures and precipitates (Heinz bodies). Phagocytic removal of Heinz bodies --> bite cells.
Howell-Jolly bodies patients with functional hyposplenia or asplenia. Basophilic nuclear remnants in RBCs.
Pappenheimer bodies Siderocytes containing basophilic granules of iron in sideroblastic anemias.
α-thalassemia α-globin gene deletion -Normal is αα/αα. α-thalassemia minima - (α α/α –) - No anemia (silent carrier) - α-thalassemia minor - α –/α –; trans) or (α α/– –; cis) - Mild microcytic, hypochromic anemia; cis deletion may worsen outcome for the carrier’s offspring (– –/– α) - - Hemoglobin H disease (HbH); excess β-globin forms β4 - Moderate to severe microcytic hypochromic anemia
Hydrops fetalis (– –/– –) Hemoglobin Barts disease; no α-globin, excess γ-globin forms γ4
β-thalassemia Point mutations in splice sites and promoter sequences β thalassemia minor (heterozygote): - β chain is underproduced. - Usually asymptomatic. Diagnosis confirmed by HbA2 (> 3.5%) on electrophoresis. β thalassemia (homozygote) -β chain is absent severe - microcytic, hypochromic anemia -target cells - increased anisopoikilocytosis - blood transfusion (2° hemochromatosis) Marrow expansion (“crew cut” on skull x-ray) --> skeletal deformities (eg, “chipmunk” facies). - Extramedullary hematopoiesis - hepatosplenomegaly. - risk of parvovirus B19–induced aplastic crisis. - HbF (α2γ2), HbA2 (α2δ2).
Orotic Aciduria Autosomal recessive. Inability to convert orotic acid to UMP (de novo pyrimidine synthesis pathway) - because of defect in UMP synthase. failure to thrive, developmental delay, megaloblastic anemia refractory to folate and B12. No hyperammonemia
Anemia of chronic disease Inflammation (eg, IL-6 --> increase in hepcidin---> decreasing the release of iron from macrophages and iron absorption from gut Hepcidin is released by liver, inhibits iron transport; - binds ferroportin on intestinal mucosal cells and macrophages, < iron, < TIBC, > ferritin.
Cryoprecipitate Contains fibrinogen, factor VIII, factor XIII, vWF, and fibronectin used for Coagulation factor deficiencies involving fibrinogen and factor VIII
Langerhans cells histocytosis proliferative disorders of Langerhans cells. -Cells express S-100 (mesodermal origin) and CD1a - Presents in a child as lytic bone lesion skin rash - recurrent otitis media, with a mass involving the mastoid bone functionally immature - do not effectively stimulate primary T cells via antigen presentation
myeloproliferative disorders -JAK2 MUTATIONS Primary polycythemia (vera) - >RBC , <EPO, > WBC, >platelet , <ESR -intense itching after hot shower. - episodic blood clots in vessels of extremities --> erythromelalgia (severe, burning pain, red-blue coloration) essential thrombocythemia -massive proliferation of megakaryocytes and platelets. - bleeding and thrombosis. Blood smear shows increased number of platelets, - may be large or abnormally formed . - Erythromelalgia may occur myeloFIbrosis -Obliteration of bone marrow with FIbrosis due to FIbroblast activity. - massive splenomegaly - “teardrop” RBC
Created by: beccao