Hematology Flashcards

1
Q

What is anemia

A

Reduction own RBC mass or blood Hgb conc. (

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2
Q

Lab test for anemia

A

CBC w/ diff
Peripheral blood smear
Reticulocyte count

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3
Q

Normocytic normochromic anemia

A

Anemia of chronic disease

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4
Q

Microcytic hypochromic anemia

A

Fe deficiency
Thalassemia
Pb toxicity

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5
Q

Microcytic anemia

A

B12 def

Folate def

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6
Q

Signs of anemia

A

Acute: lethargy, tachycardia, pallor, irritability, poor oral intake
Chronic: Few or no sxs

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7
Q

Anemias associated w/ bone marrow failure

A

Fanconi anemia

Acquired plastic anemia

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8
Q

anemia due to inherited autosomal recessive bone marrow failure

A

Fanconi anemia

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9
Q

Clinical presentation of fanconi anemia

A

Pancytopenia
Abnormal pigmentation skin
Short stature
Skeletal malformation

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10
Q

Lab findings of fanconi

A

Thrombocytopenia/ leukopenia
Anemia
Bone marrow hypoplasia-replaced w/ fat cells (aplastic anemia)

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11
Q

Tx Fanconi

A
  • Supportive tx; Anemia, transplant for thrombocytopenia, neutropenia (may be susceptible for infection)
  • Hematopoietic stem cell transplant
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12
Q

Acquired aplastic anemia

A

peripheral pancytopenia w/ a hypo cellular bone marrow
50% cases idiopathic
Other causes: meds, toxic exposure, viruses

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13
Q

Clinical presentation of acquired aplastic anemia

A

Weakness, fatigue, pallor, frequent infections, purpura, petechiae, bleeding

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14
Q

Lab findings for Aplastic anemia

A
  • Normocytic anemia
  • Low WBC w/ neutropenia
  • Thrombocytopenia
  • Low reticulocyte count
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15
Q

Complications of aplastic anemia

A
  • Overwhelming infection

- Severe hemorrhage

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16
Q

Aplastic anemia tx

A
  • Supportive care
  • Referral
  • Stop offending agent
  • Abx
  • RBC transfusion
  • Platelet transfusion
  • Immunosuppressants
  • HSCT
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17
Q

Most common nutritional deficiency in children, prevalently of African American and hispanic desent

A

Iron deficiency anemia

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18
Q

Signs of IDA

A

Pallor
Fatigue, irritability
Delayed motor debt
Hx of pica

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19
Q

When does screening for anemia need to be done

A

12mos of age (Hgb conc & risk factor assessment)

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20
Q

Risks for IDA

A
  • Low socioeconomic status
  • Premature/low birth weight
  • Lead exposure
  • Exclusive breast feeding beyond 4mos
  • Weaning to whole milk/foods that don’t contain iron
  • Feeding probs
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21
Q

Lab findings for IDA

A
  • Microcytic hypo chromic anemia

- Hgb

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22
Q

IDA tx

A
  • Hgb 10-11mg/dL @ 12mo visit; monitor closely, recheck Hgb in 1mo
  • Iron 6mg/kg/d, 3x qd
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23
Q

Types of megaloblastic anemia

A
  • Vit B12 def

- Folic acid def

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24
Q

Causes of Vit B12 def

A
  • Intestinal malabsorption

- Dietary insufficiency (req animal protein)

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25
Q

Causes of folic acid def

A
  • Increased folate req (rapid growth, chronic hemolytic anemia)
  • malabsorptive synd (celiac)
  • Inadequate dietary intake
  • Meds (methotrexate)
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26
Q

Clinical presentation Megaloblastic anemia

A

Pallor
Glossitis
*Older can w/ Vit B12 = parenthesis, weakness, unsteady gait, decreased vibratory sense/proprioception

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27
Q

Lab findings Megaloblastic anemai

A

Elevate MCH & MCV
Low folic acid +/- vit B12
Hyper segmented neutrophils
Large oval RBCs (macro ovalocytes)

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28
Q

Tx megaloblastic anemia

A

Vit B12/Folate supplements

Must treat Vit B12 Def or neurological def will be permanent

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29
Q

Which diseases are examples of congenital hemolytic anemias

A
  • Hereditary spherocytosis
  • Thalassemia
  • Sickle cell disease
  • G6PD
  • Pb poisoning
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30
Q

Hereditary spherocytosis

A
  • Red cell membrane defect
  • Pt-Jaundice, splenomegaly, gall stones
  • Lab-spherocytes on peripheral smear
  • increased osmotic fragility
  • Dx-Supportive care +/- RBC transfusion
  • possible splenectomy
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31
Q

Thalassemia

A
  • alpha or beta, more deletions = more severe, microcytic hypochromic
  • Lab-Hgb Electrophoresis
  • Rx-Fe monitoring/chelation, splenectomy, HSCT (beta)
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32
Q

Sickle cell

A
  • Homozygous HbSS, vasoocclusion=pain
  • Pt-splenomegaly, asplenia, increased risk of sepsis, delayed puberty
  • Hgb Electrophoresis
  • Rx-Avoid precipitating factors, supportive, hyrdoxyurea, stem cell transplant
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33
Q

Disease characterized by howell jolly bodies

A

sickle cell anemia

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34
Q

G6PD def

A
  • X linked recessive red cell enzyme, causes hemolytic anemia, highest among African, Medit, Asian. episodic hemolysis due to exposure to oxidant stress, drugs and food substances
  • Pt-neonatal jaundice, hyperbilirubinemia, pallor
  • Bite like deformities and Heinz bodies on peripheral smear
  • Tx-supportive, avoidance of certain food/drugs
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35
Q

Pb poisoning

A
  • Lead based paint exposure (old home), hemolytic, normocytic
  • Labs-basophilic stippling
  • Tx-chelation
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36
Q

Disease characterized by heinz bodies

A

G6PD def

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37
Q

Disease characterized by Basophilic stippling

A

Lead poisoning

38
Q

Two types of polycythemia

A

Primary

Secondary

39
Q

Primary polycythemia

A
  • Congenital; many cells = risk of thrombosis, only RBCs affected
  • High Hgb (27g/dL)
  • splenomegaly, plethora (ruby red appearance), lethargy, HA
  • Rx: Phlebotomy
40
Q

Secondary polycythemia

A
  • Occurs in response to hypoxemia (cyanotic congenital heart disease, chronic pulmonary disease)
  • Rx: Treat underlying condition, phlebotomy
41
Q

What tests do we do when we are concerned about a bleeding disorder

A
  • CBC (platelet count)
  • Peripheral smear
  • PT/INR, aPTT
  • Bleeding time
42
Q

Normal platelet count

A

150K-400K

43
Q

Platelet count that indicates spontaneous bleeding

A
44
Q

What does PT (prothrombin time) measure

A

Extrinsic and common pathway (I, II, V, VII, tissue factor)

45
Q

What does PTT/aPTT (activated partial thromboplastin time) measure

A

Intrinsic and common pathway (I, II, V, VIII, IX, X, XI, XII)

46
Q

INR measures?

A

used to monitor warfarin (coumadin)

47
Q

Bleeding time measure?

A

Platelet dysfunction (time for hemostasis)

48
Q

in what diseases is bleeding time prolonged

A

VWB, severe thrombocytopenia

49
Q

What is the most common acute bleeding disorder of childhood

A

Idiopathic thrombocytopenia purpura (ITP)- 2-5yo (post viral infection)

50
Q

Pathophysiology of ITP

A

immune mediated attack of its own platelets

51
Q

How does ITP present

A
  • Petechiae
  • Ecchymosis
  • Epistaxis
52
Q

ITP lab findings

A
  • Thrombocytopenia
  • Normal WBC
  • Normal Hgb
  • PT and aPTT normal
53
Q

ITP diagnosis

A

dx of exclusion

54
Q

ITP tx

A
  • Avoid NSAIDs/Asprin
  • Bleeding precautions
  • Prednisone
  • IVIG
  • Splenectomy
  • Observe in asymptomatic chn
55
Q

Most common inherited bleeding disorder

A

Von Willebrand disease

56
Q

Pathophysiology of VWD

A

impaired action of or decreased level of VWF = impaired platelet adhesion to endothelium. 3 types; Type 1 most common

57
Q

Clinical presentation of VWD

A
  • prolonged bleeding time from mucosa; epistaxis, menorrhagia, GI
  • Easy bruising
58
Q

Lab findings of VWD

A
  • Normal PT
  • Prolonged/norm aPTT
  • Norm decreased factor VIII
  • Norm/decreased VWF
  • prolonged bleeding time (diff than Hemophilia)
59
Q

Tx VWD

A
  • Desmopressin

- VWF replacement therapy

60
Q

What is the most common hemophilia

A

Hemophilia A (factor VIII def)

61
Q

Christmas disease

A

Hemophilia B (factor IX)

62
Q

Is hemophilia X linked

A

Yes, M>F

63
Q

Clinical presentation of Hemophilia

A
  • Bleeding
  • Mild: bleed in response to trauma/injury, not clinically apparent till later in life
  • Severe: sever bleeding, spontaneous bleeding, earlier age of first episode
64
Q

Common site of bleeding in hemophilia

A

Joints and muscles (hemarthrosis)

65
Q

Lab findings of hemophilia

A
  • Normal platelet count, PT, bleeding time
  • Prolonged aPTT
  • Normal VWF
66
Q

Tx for hemophilia

A
  • Desmopressin (hemophilia A)

- Factor replacement (VIII and IX)

67
Q

What diseases are acquired bleeding diseases

A
  • DIC
  • Liver disease
  • Kidney disease
68
Q

Acquired bleeding syndrome xtized by hemorrhage and microvascular thrombosis

A

DIC (disseminated intravascular coagulation)

69
Q

What triggers DIC

A
  • sepsis
  • trauma/tissue injury
  • malignacies
70
Q

Clinical manifestations of DIC ***

A
  • Shock
  • Diffuse bleeding tendency
  • Evidence of thrombotic lesions
71
Q

Lab findings for DIC

A
  • Decreased platelet count and fibrinogen
  • Prolonged aPTT and PT
  • Elevated D-dimer and fibrin degradation pdts
72
Q

Tx for DIC

A
  • Identification of triggering event
  • Replacement therapy
  • Anticoagulation therapy`
73
Q

Acquired bleeding disorders

A
  • Vitamin K deficiency

- Liver disease

74
Q

Where is prothrombin, fibrinogen and factors V, VII,IX, X, XII and XIII made

A

Liver

75
Q

Which factors are dependent on vitamin K

A

II, VII, IX, X (depressed neonatal activity of Vit K dependent factors)

76
Q

Lab findings for acquired bleeding disorders

A

Normal platelet count in Vit K def and Normal-low in liver disease
Prolonged PT, aPTT

77
Q

Tx for acquired bleeding disorders

A
  • Treat underlying condition

- vit K at birth

78
Q

Inherited thrombotic disorders

A
  • Protein C def
  • Protein S def
  • Antithrombin Def
  • Factor V leiden mutation
79
Q

Thrombotic disorder in which activated protein C inactivates activated factors V and VII

A

Protein C deficiency (Tx w/ Heparin) -

80
Q

Danger in protein C def

A

warfarin induced necrosis

81
Q

What is the role of protein S

A

Co-factor for protein C that facilitates the action of activated protein C

82
Q

Factor V leiden mutation pathophys

A

point mutation that makes factor V resistant to inactivation by activated protein C`

83
Q

What does factor V leiden mutation increase the risk of

A

-VTE (2-7fold), 35 fold in heterozygous taking oral contraceptives, 80 fold in homozygous

84
Q

pathophys of antithrombin def

A

Inhibitor of thrombin (efficiency of heparin may be diminished)

85
Q

Tx for thrombotic disordera

A
  • Anticoagulation prophylaxis

- Anti coat agents (unfractionated heparin, low molecular weight heparin, warfarin)

86
Q

How long should you treat an adult post first VTE episode

A

3mos

87
Q

Inflammation of deposits of IgA complexes in the GI, kidneys, skin (small vessel vasculitis) often presenting with hematuria

A

Henoch-Schonlein purpura (HSP)

88
Q

What is the most common type of small vessel vasculitis

A

HSP

89
Q

Clinical presentation of HSP

A
  • Palpable Purpura
  • arthritis/arthralgia
  • Abdominal disease
  • Renal disease
90
Q

Lab findings of HSP

A
  • Normal/elevated platelet count
  • Hematuria (UA)
  • Hemoccult
  • Elevated serum IgA
  • Elevated antistreptolysin O
91
Q

Tx HSP

A

-Supportive (good prognosis)