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45 Cards in this Set
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hereditary spherocytosis
Anemia Type Clincal Manifestation Histology |
Inherited Hemolytic Anemia
defective ankyrin causes defect in spectrin- makes them osmotically fragile- easily damaged RBC-sequestered by the spleen Clinically- splenomegaly, anemia, and jaundice, and decreased life span of erythrocytes- infection can tip over the edge Histology- Target cells-round RBC, occational shitocytes |
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hypersplenism
Anemia Type Clincal Manifestation Histology |
Acquired Hemolytic Anemia
Often due to Portal Hypertension, etc Histology- Shistocytes |
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Iron Deficiency
Anemia Type Clincal Manifestation labs |
Microcytic Anemia
Clincal- weakness, fatigue, angular stomatitis, and koilonychias, or spooning of the nails Labs- increase TIBC and decreased Ferritin Histology- Hypochromic and microcytic |
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Beta thalassemia
Anemia Type Clincal Manifestation Histology |
Microcytic Anemia
over 300 mutations-alteration of splicing machinery Clincally- Minor (N/+ or N/0)= asymptomatic-watch infection and drugs Intermediate (N/O, +/0)= mild/severe-watch infection and drugs Major (+/0, 0/0)= tranfusion dependant Histology- anisocytosis and poikilocytosis (altered size and shape of RBCs), hypochromia, target cells, and fragmented red cells |
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sickle cell anemia
Anemia Type Clincal Manifestations Histology |
Inherited Hemolytic Anemia
Abnormal hemoglobin-SS- abnormal shaped RBC-sickled- sequestered by the spleen Clincally- Microvascular infarct-pain crisis, dactylitis, anemia due to decreased Hb carrying capacity- esp with dehydration Histology- hyperchromic sickle cells |
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Alpha Thalassemia
Anemia Type Clincal Manifestation Histology |
Microcytic Anemia
Deletion of 1-2 are asymptomatic 3 Deletions- hemoglobin H disease- moderate anemia 4 Deletions- Hydrops Fetalis |
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megaloblastic anemia
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Vit B12- anemia plus Dorsal Column and UMN (+ methymalonate)
Folate- anemia only Chemo Myelodysplastic disorders |
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microcytic anemia
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Iron Deficiency- increase TIBC and decreased Ferritin
Anemia of Chronic Inflammation- increased Fe storage Sideroblastic- rings in bone marrow with dimorphic smear Thalassemia- normal labs |
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acquired hemolytic anemia
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Hypersplenism
DIC,hemolytic uremic syndrome, SLE, TTP Drug Induced- Aldomet/Sulfa/Hapten Alloimmune-transfussion Autoimmune- Cold/Warm anitbody |
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inherited hemolytic anemia
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Sphericytosis
Ellipsocytosis Pyropoikilocytosis Sickle Cell G6PD |
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Normocytic Anemia
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Metabolic- Renal Failure, Pregnancy (ficticious-increased plama volume)
Endocrine- Decreased Testosterone, hypothroid, adrenal failure Marrow Replacement- granulomatous or metastatic breast, lung, or prostate cancer Pancytopenia- Aplastic, Acute Leukemia |
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G6PD Deficency
Anemia Type Clincal Manifestations Histology |
Inherited Hemolytic Anemia
Manifests when given Oxidatative Drugs Histology- Heinz Body- Hb ppt- bite cells |
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Anemia of chronic inflammation
Anemia Type Labs |
Normocytic (with elevated Retic) to eventully Microcytic (with reduced Retic) when Fe runs out
Elevated cytokines- reduced erythrocyte production and eventually low Fe and high storage- but giving Fe doesn't fix the underlying problem |
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Warm autoimmune anemias
Anemia Type |
Acquired hemolytic Anemia- + Coombs
IgG mediated- sent to spleen for destruction Idiopthic or assoc. with lymphomas, leukemias, SLE, drug reactions, or other neoplastic disorders Splenomegaly Histology- "bite cells" RBC and shistiocytes |
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Cold autoimmune anemias
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Acquired hemolytic Anemia- + Coombs
Infection or Idiopathic IgM mediated and Complement |
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Drug induced hemolysis Mechanisms
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1. antibodies binding to drug bound to membrane proteins
2. immune complex of drug and antibody that binds complement to the red cell membrane. 3. autoantibody 4. oxidant hemolysis of G6PD deficient red cells |
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immune thrombocytopenic purpura (ITP)
Chronic Vs Acute Clincal Manifestations Histology |
IgG autoantibodies to platelets- most commonly to Gp IIb/IIIa on the platelet surface- macropahges destroy the platelets- Thrombocytopenia
Acute= Kids-usually viral Chronic= Older Women-autoimmune Clincally- increased menstrual bleeding, Petechiae, ecchymoses, Nosebleeds, extensive hemorrhage after minor trauma, gingival bleeding, subarachnoid hemorrhage, stroke (hemorrhagic) Histology- decreased platelets, RBC inclusions, BM-many megakaryocytes |
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Hemophelia B
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Factor IX deficiency "Christmas disease"
presents clinically identical to hemophilia A Prolonged PTT everything else normal |
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Hemophilia A
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Factor VIII deficiency
X-linked recessive characterized by hemarthroses Labs- component of the intrinsic pathway,leads to a prolonged PTT with normal BT, platelet count, and PT |
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prolonged PTT
Definition Conditions that result in it |
Coagulation Test of the intrinsic pathway
Defect in: 1. factors VIII (hemophilia A) 2. IX (hemophilia B), 3. XI, or XII (rarely) 4. severe vWD 5. Heparin 6. Lupus anticoagulants |
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von Willebrand’s disease
Type 1 Type 2A Type 2B Type 2M Type 2N Type 3 DDAVP contraindications? |
Type 1= reduced amount made- Autosomal Dominant- Tx with DDAVP
Type 3= None made (no vWF Ag)- Tx with replacement- Humate/Cryo Type 2A= Large Multimers- Tx replacement Type 2B= increased binding to Gp1b (increased RIPA)- Tx replacement * NO DDAVP Type 2M= defect in Gp1b (decreased RIPA)- Tx replacement, similar to Bernard Soulier (defect on platelet) Type 2N= unstable VIII carrier- looks like Hemophilia- Tx replacement |
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Hemolysis Labs
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1. Isolated increased Indirect Bilirubin- *best test
2. Elevated Retic % with Anemia 3. Breath CO- test- Hb breakdown 4. elevated urobilinogen in urine 5. hemosiderinuria, hemoglobinemia, or hemoglobinuria= severe hemolysis intravascularly 6.Increased lactic dehydrogenase (LDH) - not specific 7. Decreased haptoglobins- all bound to lysed Hb 8. Decreased Measured RBC Survival- not specific |
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Platelet Vs Coagulation Bleeding
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Platelet= mucocutaneous & subcutaneous bleeding- bruising, petechiae, etc
Coagulation Bleeding= deep tissue bleeds, hemarthralgias |
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Prothrombin time (PT)
Definition Disorders that result in it |
Extrinsic Pathway
Time for plasma to clot in presence of exogenously added source of tissue thromboplastin (aka tissue factor, Factor III) and Ca++. Defects in: 1. vitamin K-dependent factors(2,7,9,10,prot c and s) 2. Liver disease 3. DIC 4. oral anticoagulants (Coumadin) |
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Bleeding time (BT)
Definition Defects in it |
Time for standard skin puncture to stop bleeding.
Becoming obsolete b/c of PFA test Tests Primary hemostasis Defects- 1. platelet defects 2. vWD |
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coumadin skin necrosis
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If coumadin is given alone to a Protein C and/or Protein S deficient patient, the little remaining Protein C/S will be depleted (a pro-coagulant effect) before the levels of II, VII, IX and X decrease Get exacerbation of the hypercoaguable state -cause microthrombi that form in the capillaries of the skin.
Give heparin First!- counteract transient state of hypercoaguability. |
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Causes of B12 Deficiency
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1. pernicious anemia
2. chemotherapy, some antivirals, methotrexate, and hydroxyurea 3. Crohn’s disease 4. tapeworm infestation |
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reticulocyte Count
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Retics <2%: decreased production i.e. hypoproliferative (eg: bone marrow failure)
Retics >2%: increased loss, bone marrow OK (eg: bleeding = most common cause of low HCT) * reticulocyte percentage must be normalized for patients with anemia because their HCT is already too low |
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Coomb’s Test
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Test for antibody or complement is present on the RBC membrane
+ if: 1. Alloimmune- Blood tranfusion 2. Autoimmune- Warm (IgG)/Cold (IgM/Complement) Antibodies 3. Drug Induced Antibdies |
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thrombotic thrombocytopenic purpura (TTP)
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Absent or severely reduced activity of ADAMTS-13 due to auto-antibody formation-Non-cleaved ULVWF multimers induce platelet adhesion and aggregation
* Use up platelets and can't produce them fast enough- causes bleeding and clotting- can result in Fragmented Hemolytic Anemia |
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Iron Studies
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To differentiate Microcytic Anemia
Fe Deficiency- dec serum Fe, inc TIBC, 10% sat, dec Ferritin, no storage Anemia or Chronic Infl.- dec serum Fe, dec TIBC, 5-15% sat, inc Ferritin, inc storage Sideroblastic- inc serum Fe, nl TIBC, 50-100%, inc ferritin, inc storage, ring sideroblasts Thalassemia- normal labs, maybe inc serum Fe and inc ferritin * can have an inc retic count |
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disseminated intravascular coagulation (DIC)
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thrombohemorrhagic disorder -equilibrium tips towards clot formation-always a secondary manifestation
2 Types- 1. Pure consumption of platelets- Thrombocytopenia 2. Platelet and Fibrinogen activation- Thrombocytopenia and coagultion factor deficiency Labs- D Dimer inc, PT and PTT prolonged, platelets dec, and fibrinogen dec Tx- Heparin if no bleeding, cryoprecipitate if bleeding (need fibrinogen) *can cause a fragmented Hemolytic Anemia |
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Causes of Thrompocytopenia
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platelet count of <100,000
1. HIV 2. DIC 3. HUS (Uretic syndrome) 4. Heparin (HIT) 5. ITP (GpIIb) 6. TTP (ADAMS13) |
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Causes of fragmentation hemolysis
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1. DIC
2. malignant hypertension 3. TTP (thrombotic thrombocytopenic purpura) 4. HUS 5. Valves- prostheses and diseased 6. malignancy |
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pRBC
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packed RBC
Give for decreased hematocrit- to return O2 carrying capcity- NOT for volume- give saline in that case |
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FFP
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Fresh Frozen Plasma- to replace coagulation factors
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cryoprecipitate
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give to replace fibrinogen
Contains- Factor VIII, Factor XIII, fibrinogen and von Willebrand’s factor |
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NAT vs Antibody tested infections in blood supply
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NAT- HIV, HCV, West Nile
Anti-body- HBV, Syphilis, Malaria, HTLV 1-2 WBC-(leukoreduction)- CMV, EBV |
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Leukoreduction vs Irradiation
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Leukoreduction - 99.9% WBC removal- to decrease cytokine production, HLA alloimmunization, and CMV/EBV transmission
Irradiation- done in immunocompromised pts to reduce GVHD |
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Transfusion-Related Acute Lung Injury (TRALI)
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due to anti-HLA antibodies in the plasma of donors causing WBC stagnation in the lungs of recipients with HLA antigens matching the donor antibodies
*second leading cause of transfusion death |
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7 Causes of hypercoagulability
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1. Factor V Leiden mutation
2. prothrombin gene mutation (in the untranslated region) 3. elevated Lp (a) 4. decreased Protein C, S and Anti-Thrombin III 5. elevated homocyteinuria (B12/folate deficiency, MTHFR mutation) 6. Cancer 7. Elevated Factor VIII (acute phase protein) |
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Vit K dependant factors
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VII
IX X prothrombin (II) proteins S and C |
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Virchow's Triad
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1. stasis
2. vascular injury 3. hypercoagulability |
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Lupus Anticoagulants
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Anti-phosolipid antibodies- cause clotting
look for an isolated PPT |
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HIT (Heparin Induced Thrombocytopenia)
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*major cause of malpractice
50% decrease in platelet count form antibodies to platelet factor IV usually 10-14 days into Tx if 1st time Normal Mechanism= increase Anti-Thrombin III acivity (so don't give it to your ATIII deficient patients) |