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45 Cards in this Set

  • Front
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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
hypersplenism

Anemia Type
Clincal Manifestation
Histology
Acquired Hemolytic Anemia
Often due to Portal Hypertension, etc
Histology- Shistocytes
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
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
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
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
megaloblastic anemia
Vit B12- anemia plus Dorsal Column and UMN (+ methymalonate)
Folate- anemia only
Chemo
Myelodysplastic disorders
microcytic anemia
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
acquired hemolytic anemia
Hypersplenism
DIC,hemolytic uremic syndrome, SLE, TTP
Drug Induced- Aldomet/Sulfa/Hapten
Alloimmune-transfussion
Autoimmune- Cold/Warm anitbody
inherited hemolytic anemia
Sphericytosis
Ellipsocytosis
Pyropoikilocytosis
Sickle Cell
G6PD
Normocytic Anemia
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
G6PD Deficency

Anemia Type
Clincal Manifestations
Histology
Inherited Hemolytic Anemia
Manifests when given Oxidatative Drugs
Histology- Heinz Body- Hb ppt- bite cells
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
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
Cold autoimmune anemias
Acquired hemolytic Anemia- + Coombs
Infection or Idiopathic
IgM mediated and Complement
Drug induced hemolysis Mechanisms
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
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
Hemophelia B
Factor IX deficiency "Christmas disease"
presents clinically identical to hemophilia A
Prolonged PTT everything else normal
Hemophilia A
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
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
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
Hemolysis Labs
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
Platelet Vs Coagulation Bleeding
Platelet= mucocutaneous & subcutaneous bleeding- bruising, petechiae, etc
Coagulation Bleeding= deep tissue bleeds, hemarthralgias
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)
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
coumadin skin necrosis
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.
Causes of B12 Deficiency
1. pernicious anemia
2. chemotherapy, some antivirals, methotrexate, and hydroxyurea
3. Crohn’s disease
4. tapeworm infestation
reticulocyte Count
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
Coomb’s Test
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
thrombotic thrombocytopenic purpura (TTP)
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
Iron Studies
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
disseminated intravascular coagulation (DIC)
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
Causes of Thrompocytopenia
platelet count of <100,000
1. HIV
2. DIC
3. HUS (Uretic syndrome)
4. Heparin (HIT)
5. ITP (GpIIb)
6. TTP (ADAMS13)
Causes of fragmentation hemolysis
1. DIC
2. malignant hypertension
3. TTP (thrombotic thrombocytopenic purpura)
4. HUS
5. Valves- prostheses and diseased
6. malignancy
pRBC
packed RBC
Give for decreased hematocrit- to return O2 carrying capcity- NOT for volume- give saline in that case
FFP
Fresh Frozen Plasma- to replace coagulation factors
cryoprecipitate
give to replace fibrinogen
Contains- Factor VIII, Factor XIII, fibrinogen and von Willebrand’s factor
NAT vs Antibody tested infections in blood supply
NAT- HIV, HCV, West Nile
Anti-body- HBV, Syphilis, Malaria, HTLV 1-2
WBC-(leukoreduction)- CMV, EBV
Leukoreduction vs Irradiation
Leukoreduction - 99.9% WBC removal- to decrease cytokine production, HLA alloimmunization, and CMV/EBV transmission
Irradiation- done in immunocompromised pts to reduce GVHD
Transfusion-Related Acute Lung Injury (TRALI)
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
7 Causes of hypercoagulability
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)
Vit K dependant factors
VII
IX
X
prothrombin (II)
proteins S and C
Virchow's Triad
1. stasis
2. vascular injury
3. hypercoagulability
Lupus Anticoagulants
Anti-phosolipid antibodies- cause clotting
look for an isolated PPT
HIT (Heparin Induced Thrombocytopenia)
*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)