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Flashcards in Hematology Deck (97)
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61

Labs in PNHb?

CD55/59 negative RBCs

62

Treatment of PNHb?

Eculizumab (terminal complement inhibitor)

63

Sickle Cell Anemia Defect

Point mutation - glutamic acid --> valine (neutral) --> extravascular and intravascular hemolysis

64

Complications in sickle cell

- Crew cut on skull X-ray
- Aplastic crisis (B19)
- Autosplenectomy --> increased risk of infection by encapsulated organisms (S. pneumo, H. flu)
- Splenic infarct/sequestration crisis
- Salmonella osteomyelitis
-Painful crises (dactylitis, priapism, acute chest syndrome, avascular necrosis, stroke)
- Renal papillary necrosis and microhematuria

65

Sickle cells CoD?

- Kids: encapsulated respiratory infection
- Adults: acute chest syndrome

66

Sickle cell rx?

Hydroxyurea (increases HbF), hydration

67

Warm AI Hemolytic Anemia

IgG
- Chronic
- SLE, CLL, certain drugs (alpha-methyldopa, penicilin)

68

Cold AI Hemolytic Anemia

IgM and complement
- Acute
- Triggered by cold
- Seen in CLL, mycoplasma pneumonia, mono

69

AI Hemolytic Anemia lab?

+ Coombs

70

Microangiopathic Anemia

- Obstructed/narrowed vessel lumina
- DIC, TTP/HUS, SLE, malignant HTN
- SCHISTOCYTES

71

Describe Parvovirus B19

SS nonenveloped DNA
linear and small

72

Acute Intermittent Porphyria E?

Porphobilinogen Deaminase
- Increased porphobilinogen, S-ALA, coporphobilinogen in urine

73

Presentation of acute intermittent porphyria?

- Painful abdomen
- Port wine colored urine
- Polyneuropathy
- Psych disturbances
- Precipitated by drugs, alcohol, starvation

74

Rx for acute intermittent porphyria?

Glucose and heme --> inhibits ALA-synthase

75

Porphyria cutanea tarda E?

Uroporphyrinogen decarboxylase
- Urophoryrin elevated in urine (tea-colored urine)

76

Porphyria cutanea tarda presentation?

Blistering cutaneous photosensitivity

77

Labs in Hemophilia?

Increased PTT

78

Treatment for hemophilia?

Desmopressin (vWF and factor VII)
- Factor VIII concentrate (A)
- Factor IX concentrate (B)
- Factor XI concentrate (C)

79

Vitamin K deficiency Labs?

Increased PT and PTT

80

Difference between cogulation factor inhibitor or hemophilia?

PTT does NOT correct upon mixing normal plasma with patient plasma due to inhibitor (vs. hemophilia A)

81

Liver failure's effect on hemotology?

- Decreased production of coag factors
- Decreased activation of Vit K
- Followed using PT

82

Bernard-Soulier Syndrome
- QUALITATIVE Platelet Prob

- Decreased GpIb --> defect in platelet-to-vWF adhesion
- Large platelets ("big suckers")
- Increased BT

83

Glanzmann Thromasthenia
- QUALITATIVE Platelet Prob

- Decreased GpIIb/IIIa --> defect in platelet-to-platelet aggregation
- Increased BT

84

Hemolytic Uremic Syndrome Labs
- QUANTITATIVE platelet prob (decreased PC and BT)

- Thrombocytopenia, microangiopathic hemolytic anemia, acute renal failure
- KIDS w/ DIARRHEA (STEC O157:H7)
- Rx: plasmapharesis

85

Immune Thrombocytopenia
- QUANTITATIVE platelet prob (decreased PC and BT)

Anti-GpIIb/IIIa Ab --> splenic mac consumption of platelet-Ab complex
- Viral illness
- Increased megakaryocytes
- Rx: steroids, IVIG, splenectomy

86

Thrombotic Thrombocytopenic Purpura
- QUANTITATIVE platelet prob (decreased PC and BT)

- Inhibition/deficiency of ADAMTS 13 = vWF metalloprotease --> decreased degradation of vWF --> large vWF multimers --> increased platelet adhesion --> increased platelet aggregation and thrombosis

87

Labs on TTP?

Schistocytes, increased LDH

88

Sx of TTP?

Pentad: neuro sx + renal sx + fever + thrombocytopenia + microangiopathic hemolytic anemia (schistocytes)

89

von Willebrand Disease Labs

- Increased BT (decreased platelet-to-vWF adhesion)
- Increased PTT (intrinsic, vWF carries/protects factor VIII)
- Abnormal ristocetin test (no platelet aggregation)

90

vWF Disease Genetics

AD