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Hematology Flashcards

1
Q

Microcytic Anemias (MCV <80)

A
  • Thalassemia
  • Iron Deficiency
  • Lead poisoning
  • Sideroblastic anemia
  • Anemia of chronic disease
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2
Q

Normocytic anemia (MCV 80-100)

A
  • Anemia of chronic disease
  • Hemolytic anemia
  • Hemorrhage
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3
Q

Macrocytic anemia (MCV >100)

A
  • Folate deficiency
  • Vit B12 deficiency
  • Liver disease
  • Alcohol abuse.
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4
Q

Iron deficient anemia

Pt

Dx

Management

A
  • Pt
    • fatigue, SOB, tachycardia, pallor.
    • Ice pica.
    • restless leg syndrome
    • angular cheilitis
  • Dx
    • low H&H, low MCV, low MCH, MCHC.
    • Iron study: Low iron, High transferrin, low ferritin, low % sat.
  • Tx
    • iron supplementation
    • if >50, perform colonoscopy to rule out GI bleed.
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5
Q

Lead Poisoning

A
  • Pt
    • microcytic anemia.
    • abd pain, constipation
    • joint, muscle pain.
    • HA, peripheral neuropathy.
  • Dx
    • Low H&H, low MCV
    • High serum Iron
    • Basophilic stippling on peripheral smear
  • Tx
    • eliminate source of exposure
    • Chelation
      • Adults: EDTA, succimer
      • Children: dimercaprol
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6
Q

Sideroblastic anemia

A
  • Etiology
    • genetic.
    • EtOH abuse or isoniazide
  • Pt
    • microcytic anemia, fatigue, pallor, SOB
  • Dx
    • Bone marrow biopsy w/ ringed sideroblasts.
    • basically the only blood findings will be microcytic anemia.
  • Tx
    • ​Genetic: Vit B6
    • Acquired: stop EtOH, isoniazide.
      • RBC transfusions, EPO
      • Deferoxamine, phlebotomy
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7
Q

Alpha Thalassemia

A
  • Minima
    • 1 globin allele
    • Asymptomatic
  • Minor (trait)
    • 2 globin allele
    • asymptomatic
  • Hemoglobin H disease
    • 3 allele.
    • microcytic anemia, chronic hemolysis
  • ​Hydrops fetalis
    • 4 allele.
    • Hgb Bart (4 gamma globins)
    • fetal edema and intrauterine death. Mimics a Lt shift.
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8
Q

Beta thalassemia

A
  • Minor
    • 1 allele
    • low beta gloin.
    • High Hgb A2 (alpha, delta)
    • Minimal disease
  • Major
    • 2 allele
    • (+) Hgb F (alpha, gamma + Hgb A2
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9
Q

Sickle Cell Disease

Genetics

Pt

A
  • Genetics
    • Glu to Val mutation at position 6 of chr 11.
    • AR inheritance.
  • Pt
    • Acidosis, hypoxia, dehydration cause cell sickling which leads to hemolysis and vaso-occlusive crisis.
    • acute chest syndrome
    • stroke
    • Dactylitis (finger swelling)
    • bone infarction, osteonecrosis
    • Priapism
    • Splenic sequestration –> auto-splenectomy
    • Parvovirus B19 leads to aplastic crisis (where all cell lines are low).
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10
Q

Sickle Cell disease

Dx

Tx

A
  • Dx
    • anemia
    • sickled RBC peripheral smear
    • Hgb electrophoresis -> Hgb S + Hgb F
    • Skull xray = hair on end appearance
  • Tx
    • IVF, supplemental O2, opioid analgesics
    • Folic acid to supplement RBC production
    • Hydroxyurea
    • Immunize: pneumococcal, H. influenza B, Meningococcal, influenza.
  • < 5: prophylactic penicillin.
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11
Q

Diagnosis of Hemolytic anemia

A
  • Low H&H
  • Normal MCV
  • High indirect bili
  • High LDH
  • Low serum haptoglobin.
  • High retic count.
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12
Q

Causes of Extrinsic Hemolysis

A
  • Microangiopathic anemia
    • RBC death by narrow lumen passage.
    • (+) schistocytes
  • Macroangiopathic anemia
    • Mechanical forces shear RBC.
  • RBC infection
    • malaria, babesiosis
  • Autoimmune
    • (+) direct coombs test.
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13
Q

Autoimmune hemolytic anemia

A
  • Cold agglutinins
    • Ab to RBC < 4degrees.
    • IgM. Seen with EBV, mycoplasma infection.
  • Warm agglutinins
    • Ab to RBC at normal body temp.
    • IgG. HIV, EBV, Lupus, Cancers.
  • Drug-induced
    • cephalosporin
    • penicillin
    • NSAID
    • methyldopa.
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14
Q

Treatment of autoimmune hemolytic anemia

A
  • Cold agglutinins
    • avoid cold temps
  • Warm agglutinins
    1. Steroids
    2. Rituximab (monoclonal antibody against B cells)
    3. Splenectomy
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15
Q

Hereditary spherocytosis

A

Genetic defect in RBC membranes and cytoskeleton

  • Pt
    • splenomegaly, jaundice
    • high risk aplastic anemia w/ parvovirus B19 infection
  • Dx
    • anemia w/ reticulocytosis and high MCHC.
    • (+) spherocytes.
    • (+) osmotic fragility test
  • Tx
    • Folic acid 1mg QD
    • RBC transfusion if severe anemia
    • Splenectomy –> (+) Howell-Jolly bodies.
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16
Q

G6PD deficiency is exacerbated by what?

A

Spleen Purges Nasty Inclusions From Damaged Cells

  • Sulfonamides
  • Primaquine
  • Nitrofurantoin
  • Isoniazide
  • Fava bean
  • Dapsone
  • Chloroquine.
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17
Q

Diagnostic tests that indicate G6PD deficiency

A
  • (+) Heinz bodies: precipitation of oxidized Hemoglobin in RBC
  • (+) Degmacytes: deformed RBC following removal of Heinz bodies by spleen.
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18
Q

Urticarial transfusion reaction

Timing

Cause

Findings

Tx

A
  • Timing
    • during transfusion
  • Cause
    • IgE antibodies against the donor plasma
  • Findings
    • Urticaria
  • Tx
    • diphenhydramine
    • stop transfusion
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19
Q

Anaphylactic transfusion reaction

Timing

Cause

Findings

Tx

A
  • Timing
    • seconds to minutes after start
  • Cause
    • Anti IgA/IgG antibodies in patient with IgA deficiency
  • Findings
    • Shock, HoTN, angioedema, respiratory failure.
  • Tx
    • Epinephrine, IVF, O2
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20
Q

Non-Hemolytic Febrile transfusion reaction

Timing

Cause

Findings

Tx

A
  • Timing
    • 1-6hr s/p
  • Cause
    • Cytokines produced by donor cells
  • Findings
    • fever, chills, malaise
  • Tx
    • Acetaminophen
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21
Q

Acute hemolytic transfusion reaction

Timing

Cause

Findings

Tx

A
  • Timing:
    • during transfusion
  • Cause
    • ABO incompatibility
  • Findings
    • Hemolysis, fever, chills, tachycardia, tachypnea, HoTN.
  • Tx
    • aggressive supportive therapy
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22
Q

Delayed hemolytic transfusion reaction

Timing

Cause

Findings

Tx

A
  • Timing
    • 2-10 day s/p
  • Cause
    • Anti-Kidd or anti-D antibodies
  • Findings
    • Mild fever, hemolysis, high indirect bili
  • Tx
    • None
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23
Q

Aplastic Anemia

Etiology

Pt

Dx

Tx

A
  • Etiology
    • radiation exposure
    • Rx: chemotherapy, chloramphenicol, sulfonamides, phenytoin
    • parvovirus B19 infection
  • Pt
    • Anemia, Leukopenia, Thrombocytopenia
  • Dx
    • (+) anemia, leukopenia, thrombocytopenia (>50% decrease of normal baseline)
    • Bone marrow Bx = hypocellular and fat infiltration
  • Tx
    • bone marrow transplant
    • Immunosuppressants.
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24
Q

B12 deficiency

A
  • Etiology
    • pernicious anemia.
    • Gi bypass/resection (terminal ileum)
    • strict vegans w/ no meat/dairy intake.
  • Pt
    • anemia
    • dementia, Dorsal column dysfunction
    • atrophic glossitis (tongue inflammation w/ loss of papillae)
  • Dx
    • MCV > 100
    • low B12.
    • High MMA, High Homocysteine
  • Tx
    • IM vit B12 injection
    • oral B12 supplements.
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25
Folate deficiency
* Etiology * malabsorption: alcoholism, no leafy greens * Pt * anemia. * atrophic glossitis * **NO neurological deficits.** * Dx * MCV\> 100 * **MMA normal** * Tx * folate supplements * evaluate for concurrent B12 deficiency
26
Findings of Thrombocytopenia PT Labs
* Pt * Epistaxis * Mucosal bleed * Bruising * Petechiae * Purpura * Labs * Plt \< 150,000 * Prolonged blled time * Normal PT, PTT/
27
Immune thrombocytopenia (ITP)
Anti-platelet IgG-antibodies * Plt often \< 50,000. PT/PTT are normal * Tx * Children = self limited, no tx * Adult * long-term = glucocorticoids * short term = IVIG. * Platelet transfusion if rapid/immediate control of bleeding required.
28
Thrombotic Thrombocytopenic purpura - hemolytic uremic syndrome (TTP-HUS) Mechanism Pt Tx
* Mechanism * ADAMTS13 deficiency leads to unregulated platelet aggregation causing thrombosis *and then microangiopathic hemolysis.* * *Children = induced via E. coli O157:H7.* * Pt * HUS * hemolysis, uremia, thrombocytopenia * TTP * hemolysis, uremia, thrombocytopenia, AMS/seizure, coma. * Tx * plasmapheresis + steroids.
29
Ddx of thrombocytopenia
* Parvovirus B19 infection * Megaloblastic anemia (B12, folate) * Alcohol induced * Splenic sequestration * HELLP.
30
Von Willebrand Disease
AD deficiency of vWF and F8 * Pt * easy bruising, mucosal bleeding, skin bleed, menorrhagia. * Dx * High PTT, high bleed time. * Plt = normal * vWF - low. ristocetin cofactor activity is low. F8 low. * Tx 1. Desmopressin (DDAVP) for acute bleed. 2. vWF concentrate if refractory 3. OCP for menorrhagia *_avoid platelet inhibitors_*
31
Hemophilias
X-linked recessive disorder * Pt * Hemarthrosis, hematuria, GI bleed, mucosal bleeds. * Dx * High PTT. * Normal PT. Check factor 8 and 9 levels/ activity * Tx * Replace F8 (a) * Replace F9 (b) * DDAVP will increase F8
32
Disseminated Intravascular Coagulation (DIC) *Causes* ## Footnote * Presentation* * Dx* * Tx*
Widespread pathologic intravascular coagulation destroying platelet and coag factors. * Causes * sepsis, trauma, OB complication, Pancreatitis, cancer, transfusion. * Pt * bleeding from wounds/surgical sites. * Hemoptysis * Venous/arterial thrombosis * HoTN, jaundice, distal extremity cyanosis. * Dx * **Low:** platelets, fibrinogen, haptoglobin. * **High:** bleed time, PT, PTT, fibrin split products, D-dimer, LDH, Bilirubin * Tx * Aggressive supportive care * Acute bleeds Tx w/ **platelet transfusion + FFP +/- RBC transfusion.**
33
**Aspirin** MOA Uses ADR
* MOA * irreversibly blocks COX-1/-2, reduces production of thromboxane-A2, reducing platelet aggregation * Use * CAD, MI, stroke * ADR * GI hemorrhage * Hyperventilation leads to resp alkalosis * Reye syndrome in children w/ viral illness.
34
ADP receptor blockers ## Footnote * MOA* * Use* * ADR*
*CLopidogrel, Ticlopidine, Prasugrel, Ticagrelor, Cangrelor* * MOA * Inhibit platelet ADP receptors; blocks expression of GpIIb/GpIIIa. * No platelet aggregation * Use * Recent MI * PVD, prevent recurrent stroke/TIA * ADR * not significant
35
GpIIb/IIIa inhibitors * MOA* * Use* * ADR*
*Abciximab, Eptifibatide, Tirofiban* * MOA * binds platelet GpIIb/IIIa, preventing aggregation * Use * Recent PCI * NSTEMI * ADR * no significant listed.
36
Dipyramidole * MOA* * Use* * ADR*
* MOA * Blocks adenosine deaminase. Vasodilates and prevents platelet aggregation * Use * Prevent recurrent stroke/TIA * ADR * HA d/t high adenosine and vasodilation
37
Heparin ## Footnote * MOA* * Use* * ADR*
* MOA * binds antithrombin and inhibits coagulation * Use * DVT prophylaxis, Acute MI, Acute PE, pregnancy anticoagulant * ADR * HIT d/t antiplatelet factor IV antibodies.
38
LMWH ## Footnote * MOA* * Use* * ADR*
*Enoxaparin, Dalteparin, Tinzaparin, Nadroparin.* * MOA * Bind antithrombin. Inactivate Factor Xa, preventing coagulation. * Use * DVT, acute PE, acute MI, pregnancy anticoagulant. * Used as alternative if (+) HIT. * ADR * some risk of HIT. very very low
39
Fondaparinux MOA Use ADR
* MOA * binds antithrombin and inhibits factor Xa * Use * DVT treatment and prophylaxis, * ADR * no significant ADR listed
40
Direct Factor Xa inhibitors ## Footnote * MOA* * Use* * ADR*
*Rivaroxaban, Apixaban, Edoxaban* * MOA * directly inhibit Factor Xa. Prevent formation of thrombin * Use * Oral anticoagulation for _A-fib, Post DVT prophylaxis_ * benefit is no requirement for INR draws to establish * ADR * none.
41
Direct thrombin inhibitors ## Footnote * MOA* * Use* * ADR*
*Dabigatran (PO), Argatroban, Bivalirudin, Desirudin (IM)* * MOA * bind thrombin * Use * A-fib, DVT prophylaxis, recent PCI, HIT * ADR * none listed.
42
Warfarin ## Footnote * MOA* * Use* * ADR*
* MOA * inhibits _epoxide reductase_ which stops formation of Vit-K dependent factors * PrC, PrS, 10, 9, 7, 2. * Use * A-fib, Mechanical heart valves, hypercoagulable states, DVT, PE. * ADR * teratogenic * drug-induced interactions. Reversal: Vitamin K (for future), FFP for immediate.
43
Common signs and symptoms of malaria
* Periodic fevers of 1-3 day intervals * Diaphoresis * HA * Arthralgias * Encephalopathy * Renal insufficiency * Pulm edema
44
Most common malaria prophylaxis
* Atovaquone - proguanil: daily * Doxycycline: daily * Mefloquine: 1-2 wk before leaving and 3-4wk after return
45
Treatment of malaria
* Atovaquone - proguanil * Mefloquine * Chloroquine * Primaquine * Quinine + doxycycline
46
Acute Lymphoblastic Leukemia _Risk factors_ _Pt_ _Dx_ _Tx_
* Risk * children 2-5. * higher risk w/ Trisomy 21. * Pt * bone pain, fever, lymphadenopathy, fatigue, recurrent infection * Dx * **PAS(+)** * **TdT(+)** * **anemia, thrombocytopenia** * Tx * Chemotherapy (vincristine + steroids)
47
Acute Myeloid Leukemia _Risk factors_ _Pt_ _Dx_ _Tx_
* Risk * Male \>65 * Pt * Fatigue, easy bruising, recurrent infections * Dx * pancytopenia, myeloblasts * **(+) Myeloperoxidase, (+) Auer rods** * Tx * Chemotherapy
48
Chronic Myeloid Leukemia _Risk Factor_ _Pt_ _Dx_ _Tx_
* Risk * 50yr adults exposed to radiation * t(9:22)/ bcr-abl translocation * Pt * fatigue, splenomegaly. * Thrombocytosis and leukocytosis * Dx * anemia, **Thrombocytosis, leukocytosis.** * Tx * Bone marrow transplant * Tyrosine-kinase inhibitor * imatinib, dasatinib, nilotinib
49
Chronic Lymphocytic Leukemia _Risk Factors_ _Pt_ _Dx_ _Tx_
* Risk * adults \>65 * Pt * asymptomatic. * fever, night sweats, fatigue, lymphadenopathy, hepatosplenomegaly * Dx * Severe leukocytosis (**WBC \> 70,000)** * **Smudge cells** * Tx * Periodic monitoring. * chemotherapy or radiation. However has poor prognosis and rapidly fatal.
50
Multiple Myeloma Pt Dx Tx
Malignant monoclonal proliferation of plasma cells (IgM) * Pt * **CRAB** * Hypercalcemia (constipation, abd pain, encephalopathy, polyuria) * Renal failure (light chain cast nephropathy, Bence jones protein) * Anemia (normocytic) * Back pain * Dx * SPEP * UPEP --\> bence jones proteins * Bone marrow Bx = high plasma cells * Tx * cytogenetic testing. * chemotherapy (lenolidomide) + marrow transplant.
51
Hodgkin Lymphoma Pt Dx Tx
B cell lymphoma with **Reedsternberg cells** w/ lymphocytes and granulocytes. * Pt * Bimodal 20's and 60's * cervical lymphadenopathy, fever, night sweats, weight loss * **Pruritus** * Dx * CT = mediastinal lymphadenopathy. (+) reed-sternberg cells. * Tx * Chemotherapy + radiation * Stem cell transplant.
52
Nodular sclerosis
* Most common form of Hodgkin lymphoma. * Nodules of lymphocytes separated by sclerotic bands of collagen. * Few R-S cells. * Good prognosis
53
Lymphocyte Rich HL
* Least common form of Hodgkin Lymphoma * Few R-S cells * Good prognosis
54
Lymphocyte-depleted Hodgkin Lymphoma
* Abundant R-S * Poor prognosis
55
Mixed cellularity Hodgkin Lymphoma
* 2nd most common form of Hodgkin Lymphoma * Mix of lymphocytes and R-S cells * NO nodules or sclerotic bands.
56
Types of Non-Hodgkin Lymphoma
* Diffuse Large B cell lymphoma * Lymphoblastic Lymphoma * Follicular Lymphoma * Burkitt Lymphoma * Small lymphocytic lymphoma * Hairy cell leukemia
57
Diffuse large B cell lymphoma
* Most common NHL * Seen in elder Men * Pt * *painless generalized lymphadenopathy.* * *fever, night sweats, wt loss*
58
Lymphoblastic lymphoma
* Most common NHL in children * Pt * painless generalized lymphadenopathy. * fever, night sweats, wt loss
59
Follicular Lymphoma
* 2nd most common NHL. **(+) t(14:18)**. notched/indented cells. * Pt * painless generalized lymphadenopathy. * fever, night sweats, wt loss
60
Burkitt Lymphoma
* t(8:14). * Starry sky appearance on Bx. * * Pt * painless generalized lymphadenopathy. * fever, night sweats, wt loss
61
Small lymphocytic lymphoma
Malignant cell which are identical to CLL. * Pt * painless generalized lymphadenopathy. * fever, night sweats, wt loss
62
Hairy cell leukemia
* lymphoma where cells have a "hairy" cytoplasmic projections * Pt * painless generalized lymphadenopathy. * fever, night sweats, wt loss
63
What is the classic presentation of Polycythemia vera?
* Visual disturbances * scotoma, ophthalmic migraine * Thrombosis * stroke, MI, angina, Budd-Chiari * Erythromelelagia * burning pain in hands and feet with erythema, pallor, cyanosis. * Pruritus (after warm bath) * hepatosplenomegaly.
64
What is the treatment for polycythemia vera?
1. Phlebotomy (Hct \<45 M. Hct \< 42 F) 2. Hydroxyura 1. add if patient is high risk for thrombosis 3. Aspirin 1. every day to reduce risk of thrombosis.

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