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
Q

Folate deficiency

A
  • 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
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26
Q

Findings of Thrombocytopenia

PT

Labs

A
  • Pt
    • Epistaxis
    • Mucosal bleed
    • Bruising
    • Petechiae
    • Purpura
  • Labs
    • Plt < 150,000
    • Prolonged blled time
    • Normal PT, PTT/
27
Q

Immune thrombocytopenia (ITP)

A

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
Q

Thrombotic Thrombocytopenic purpura - hemolytic uremic syndrome (TTP-HUS)

Mechanism

Pt

Tx

A
  • 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
Q

Ddx of thrombocytopenia

A
  • Parvovirus B19 infection
  • Megaloblastic anemia (B12, folate)
  • Alcohol induced
  • Splenic sequestration
  • HELLP.
30
Q

Von Willebrand Disease

A

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
Q

Hemophilias

A

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
Q

Disseminated Intravascular Coagulation (DIC)
Causes

  • Presentation*
  • Dx*
  • Tx*
A

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
Q

Aspirin

MOA

Uses

ADR

A
  • 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
Q

ADP receptor blockers

  • MOA*
  • Use*
  • ADR*
A

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
Q

GpIIb/IIIa inhibitors

  • MOA*
  • Use*
  • ADR*
A

Abciximab, Eptifibatide, Tirofiban

  • MOA
    • binds platelet GpIIb/IIIa, preventing aggregation
  • Use
    • Recent PCI
    • NSTEMI
  • ADR
    • no significant listed.
36
Q

Dipyramidole

  • MOA*
  • Use*
  • ADR*
A
  • MOA
    • Blocks adenosine deaminase. Vasodilates and prevents platelet aggregation
  • Use
    • Prevent recurrent stroke/TIA
  • ADR
    • HA d/t high adenosine and vasodilation
37
Q

Heparin

  • MOA*
  • Use*
  • ADR*
A
  • MOA
    • binds antithrombin and inhibits coagulation
  • Use
    • DVT prophylaxis, Acute MI, Acute PE, pregnancy anticoagulant
  • ADR
    • HIT d/t antiplatelet factor IV antibodies.
38
Q

LMWH

  • MOA*
  • Use*
  • ADR*
A

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
Q

Fondaparinux

MOA

Use

ADR

A
  • MOA
    • binds antithrombin and inhibits factor Xa
  • Use
    • DVT treatment and prophylaxis,
  • ADR
    • no significant ADR listed
40
Q

Direct Factor Xa inhibitors

  • MOA*
  • Use*
  • ADR*
A

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
Q

Direct thrombin inhibitors

  • MOA*
  • Use*
  • ADR*
A

Dabigatran (PO), Argatroban, Bivalirudin, Desirudin (IM)

  • MOA
    • bind thrombin
  • Use
    • A-fib, DVT prophylaxis, recent PCI, HIT
  • ADR
    • none listed.
42
Q

Warfarin

  • MOA*
  • Use*
  • ADR*
A
  • 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
Q

Common signs and symptoms of malaria

A
  • Periodic fevers of 1-3 day intervals
  • Diaphoresis
  • HA
  • Arthralgias
  • Encephalopathy
  • Renal insufficiency
  • Pulm edema
44
Q

Most common malaria prophylaxis

A
  • Atovaquone - proguanil: daily
  • Doxycycline: daily
  • Mefloquine: 1-2 wk before leaving and 3-4wk after return
45
Q

Treatment of malaria

A
  • Atovaquone - proguanil
  • Mefloquine
  • Chloroquine
  • Primaquine
  • Quinine + doxycycline
46
Q

Acute Lymphoblastic Leukemia

Risk factors

Pt

Dx

Tx

A
  • 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
Q

Acute Myeloid Leukemia

Risk factors

Pt

Dx

Tx

A
  • Risk
    • Male >65
  • Pt
    • Fatigue, easy bruising, recurrent infections
  • Dx
    • pancytopenia, myeloblasts
    • (+) Myeloperoxidase, (+) Auer rods
  • Tx
    • Chemotherapy
48
Q

Chronic Myeloid Leukemia

Risk Factor

Pt

Dx

Tx

A
  • 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
Q

Chronic Lymphocytic Leukemia

Risk Factors

Pt

Dx

Tx

A
  • 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
Q

Multiple Myeloma

Pt

Dx

Tx

A

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
Q

Hodgkin Lymphoma

Pt

Dx

Tx

A

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
Q

Nodular sclerosis

A
  • Most common form of Hodgkin lymphoma.
  • Nodules of lymphocytes separated by sclerotic bands of collagen.
  • Few R-S cells.
  • Good prognosis
53
Q

Lymphocyte Rich HL

A
  • Least common form of Hodgkin Lymphoma
  • Few R-S cells
  • Good prognosis
54
Q

Lymphocyte-depleted Hodgkin Lymphoma

A
  • Abundant R-S
  • Poor prognosis
55
Q

Mixed cellularity Hodgkin Lymphoma

A
  • 2nd most common form of Hodgkin Lymphoma
  • Mix of lymphocytes and R-S cells
  • NO nodules or sclerotic bands.
56
Q

Types of Non-Hodgkin Lymphoma

A
  • Diffuse Large B cell lymphoma
  • Lymphoblastic Lymphoma
  • Follicular Lymphoma
  • Burkitt Lymphoma
  • Small lymphocytic lymphoma
  • Hairy cell leukemia
57
Q

Diffuse large B cell lymphoma

A
  • Most common NHL
  • Seen in elder Men
  • Pt
    • painless generalized lymphadenopathy.
    • fever, night sweats, wt loss
58
Q

Lymphoblastic lymphoma

A
  • Most common NHL in children
  • Pt
    • painless generalized lymphadenopathy.
    • fever, night sweats, wt loss
59
Q

Follicular Lymphoma

A
  • 2nd most common NHL. (+) t(14:18). notched/indented cells.
  • Pt
    • painless generalized lymphadenopathy.
    • fever, night sweats, wt loss
60
Q

Burkitt Lymphoma

A
  • t(8:14).
  • Starry sky appearance on Bx.
    • Pt
      • painless generalized lymphadenopathy.
      • fever, night sweats, wt loss
61
Q

Small lymphocytic lymphoma

A

Malignant cell which are identical to CLL.

  • Pt
    • painless generalized lymphadenopathy.
    • fever, night sweats, wt loss
62
Q

Hairy cell leukemia

A
  • lymphoma where cells have a “hairy” cytoplasmic projections
  • Pt
    • painless generalized lymphadenopathy.
    • fever, night sweats, wt loss
63
Q

What is the classic presentation of Polycythemia vera?

A
  • 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
Q

What is the treatment for polycythemia vera?

A
  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.