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NCD final exam > Hematology > Flashcards

Hematology Flashcards

1
Q

Hemophilia

A

Definition: hereditary bleeding disorders due to deficiencies in factors VIII or IX. These result in the formation of unstable clot that can be easily dislodged, leading to excessive bleeding.

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2
Q

Hemophilia is a *** linked disorder

A

X-linked Disorders

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3
Q

Hemophilia A =

A

Factor VIII Deficiency

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4
Q

Hemophilia B

A

= Factor IX Deficiency

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5
Q

Diagnosis of Hemophilia

A

PT = normal
aPTT= PRLONGED
CBC/Plt = normal
Platlet aggregation studies - normal
mixing studies (normal)
specific factor assays (only way to differentiate A from B)

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6
Q

which diagnostic lab for hemophilia?

A

aPTT is prolonged

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7
Q

Hemophilia A

A

Most common type of hemophilia
X-linked recessive disorder resulting in deficiency in Factor VIII

S/sx: bleeding after injury or slight trauma, bleeding into muscles (pain and swelling, decreased ROM, erythema, and increased warmth), hemarthrosis, spontaneous bleeding episodes, excessive bruising/hematoma, fatigue.
Diagnosis: Factor VIII assay
PT and thrombin clotting time (TT) are normal
PTT or aPTT generally elevated but may be normal in mild disease
Treatment: none for mild signs and symptoms; infusion with Factor VIII concentrate

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8
Q

Hemophilia B

A

X-linked recessive disorder resulting in deficiency in Factor IX
Males mainly affected (females are asymptomatic carriers)
S/sx: same as hemophilia A
Diagnosis: Factor IX assay
PT and thrombin clot time are normal
aPTT generally elevated but may be normal in mild disease
Treatment: none for mild signs and symptoms; infusion of Factor IX if severe

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9
Q

Factor I

A

Fibrinogen Deficiency
Fibrinogen levels may be absent, decreased, or normal but non-functional

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10
Q

Factor II

A

Prothrombin Deficiency

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11
Q

Factor V

A

Parahemophilia, not the same as Factor V Leiden Deficiency

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12
Q

Factor VII and X

A

acquired form is more common; due to vitamin K deficiency, severe liver diseases, and anti-coagulants use such as Warfarin

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13
Q

Factor XI

A

hemophilia C, very common amongst Ashkenazi Jews, mild to moderate bleeding disorder

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14
Q

Factor XII

A

no bleeding abnormality

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15
Q

Factor VIIII

A

delayed bleeding, normal primary hemostasis; umbilical cord bleeding and intracranial hemorrhages are common

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16
Q

Von Willebrand’s Disease

A

hereditary, anormal synthesis of von Willebrand factor (vWF), which causes reduced platelet adhesion and lower serum levels of factor VIII

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17
Q

Von Willebrand’s disease

A

3x more common than hemophilia A, males/females affected the same

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18
Q

Von Willebrands disease

A

heavy menses, epistaxis, easy bruising, GI bleeding, many people never diagnosed

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19
Q

Von Willebrand Diagnostic labs

A

aPTT slightly prolonged, platlet aggregation studies (all normal except ristocetin), specific factor assays - show decreased factor VIII, vWF antigen - low.

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20
Q

Thrombocytopenia

A

decreased platelet production. aplastic anemia, vitamin B12/folate deficiency, viral infections, leukemia, drugs.

increased destruction: ITP(immune), TTP (non-immune), increased sequestration: any causes of splenomegaly, such chronic liver disease/cirrhosis

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21
Q

quantitative disorders

A

thrombocytopenia, thrombocytosis

22
Q

qualitative disorders

A

adhesion defects (vWD0), aggregation defects (DIC), secretion defects

23
Q

essential thrombocythemia

A

thrombosis may be more common than bleeding

24
Q

inadequate platelet function

A

vWD, drugs (nSAIDs), systemic disorders (lupus, uremia), inhibiting prostaglandins (multiple myeloma)

25
Q

Idiopathic/Immune Thrombocytopenic Purpura (ITP)

A

Antibodies form against platelet membrane glycoproteins and destroying platelets due to Type II hypersensitivity, which inhibits clot-formation & hemostasis
Commonly preceded by acute URI or other viral infection (antibody cross reactions), drugs and heavy metals (metabolites acting as haptens)

26
Q

ITP s/sx

A

S/sx: petechiae,, bleeding gums, nose bleeds, and intestinal bleeding, heavy menses, easy bruising, most serious complication being intracranial hemorrhage
Diagnosis:
low platelet count
Normal RBC count, WBC count, coagulation studies
History

27
Q

Acute ITP

A

Rapid onset bruising & petechiae
Primarily in children, ages 2 – 6 years
Second most common age group: young adults
Usually cross-reaction between immune response to viral infection & platelet proteins
80% cases self-limited with resolution in 1-2 months
80% of patients, their platelet count return to normal within 12 months
Lab: severe thrombocytopenia

28
Q

Chronic ITP

A

Primarily in females, ages 25 – 45 years
Associated with autoimmune conditions, such as SLE, HIV, HCV, CLL
Limited self-resolution
Up to 10% of spontaneously resolution (usually within first 6 months)
1/3 to 2/3 will reach a stable disease with first line treatment (glucocorticoids or IV immune globulin/IVIG)
Lab: degree of thrombocytopenia varies

29
Q

ITP diagnosis

A

CBC - thrombocytopenia

30
Q

Thrombotic Thrombocytopenic Purpura (TTP)

A

Rare, fatal disease. spontaneous and excessive thrombus formation. most patients develop DIC and lead to life threatening multisystem disorder

31
Q

TTP diagnosis (labs)

A

CBC, thrombocytopenia (plt count <20,000). low Hgb, low RBC,

peripheral blood smear: fragmented RBC
serum creatinine: mild elevation
serum ldh: elevated

32
Q

Disseminated intravascular coagulation (DIC)

A

Acquired
Characterized by intravascular coagulation with depletion of coagulation factors and platelets, which occludes blood supply to tissues and results in end-organ damage
Always secondary to an underlying disease:
Obstetrical complication (retained products of conception, abruptio placentae)
Infection, especially bacterial with severe sepsis
Malignancy, burns, transplant rejection

33
Q

DIC s/sx

A

S/sx: usually bleeding from at least 3 unrelated sites
Bleeding: petechiae, ecchymoses, blood oozing, hematuria, epistaxis, gingival bleeding
Thrombosis: cough and dyspnea, leg pain and swelling, organ ischemia, endocarditis
Organ dysfunction: oliguria, jaundice, confusion, delirium or coma, purpura fulminans (rare, life threatening)

34
Q

DIC diagnosis (labS)

A

CBC: thrombocytopenia
peripheral blood smear: shistocytes
PT/aPTT: prolonged
D-Dimer: elevated
Fibrinogen: decreased
Specific factor assays: decreased

35
Q

Purpura Simplex

A

most common vascular hemorrhagic disorder, women more than men. increased bruising without known trauma.

platelet count, tests of platelet function, blood coagulation, and fibrinolysis are normal.

no treatment necessary

36
Q

Senile purpura

A

affects older people, especially those with history of sun exposure

37
Q

senile purpura s/sx:

A

s/sx: dark purple ecchymosis hands and forearms. medications like anticoagulants may worsen

38
Q

Henoch-Schönlein Purpura (HSP)

A

idiopathic inflammatory disorde. immune complexes involving IgA antibodies in smaller arterioles, venules, capillaries. commonly preceeded by acute viral or bacterial infection. self-limiting.

39
Q

HSP s/sx:

A

triad of symptoms: purpuric rash, abdominal pain, joint pain.

glomerulonephritis in adutls (sequeleae)

40
Q

Polycythemia Vera

A

myeloproliferative disorder characterized by uncontrolled RBC production.

41
Q

polycythemia vera s/sx:

A

often asymptomatic, fatigue,weakness, dizziness, headaches, visual. itchiness after warm bath (aquagenic pruritus), red/warm/painful palms and soles possible digital ischemia. splenomegaly, thromboses.

42
Q

polycythemia vera labs

A

RBC, Hgb, and Hct elevated. neutrophils/ platelets may increase too. tx: phleobotomy

43
Q

secondary polycythemia

A

any elevation of Hb or Hct along with RBC incresae. no WBC or platelet increase. secondary to underlying conditions (smoking, cancers, tetralogy of fallot, chronic hypoxemia)

44
Q

Prothrombin Time (PT)

A

Prolonged in:
Vitamin K deficiency
Liver disease, including alcoholic hepatitis
Biliary obstruction
DIC
Deficiency in factor I, II, V, VII, and X
Warfarin (anti-coagulant) use

45
Q

prothrombin time (PT)

A

Measures the number of seconds it takes for a clot to form after reagents are added
Reference range: 11-13 seconds
Assess extrinsic and common pathways of the coagulation cascade
Evaluates coagulation factors: I (fibrinogen), II (prothrombin), V, VII, and X
Prothrombin is produced by the liver and its production depends on vitamin K intake and absorption

46
Q

International Normalized Ratio

A

Different reagents are used to assess PT, leading to different reference ranges.
To standardize PT results across the world, International Normalized Ratio (INR) is established (calculated based on PT results).
Used to monitor Warfarin (vitamin K antagonist) therapy.
Warfarin is prescribed for people with DVT, PE, valve replacement, atrial fibrillation, post-MI, etc. to prevent inappropriate clotting
Range should be between 2.0-3.0

47
Q

Activated Partial Thromboplastin Time (aPTT)

A

aPTT is a more sensitive version of PTT
Measures the number of seconds it takes for a clot to form after reagents are added
Reference range: 21-35 seconds
Assess intrinsic and common pathways of the coagulation cascade
Evaluates coagulation factors: all EXCEPT factor VII (assessed by PT)
Does not become prolonged until one or more clotting factors are 70% deficient

48
Q

aPTT activated in:

A

Hemophilia A and B
Vitamin K deficiency
Liver disease, including alcoholic hepatitis
DIC
Heparin therapy / Warfarin therapy

49
Q

aPTT used to monitor…

A

heparin therapy

50
Q

mixing studies

A

patient’s plasma mixed with 50:50

If clot forms (aPTT normalized/corrected), then it confirms a factor deficiency
Especially deficiencies in factors VIII, IX, XI, XII and rarely VWF
If clot fails to form (aPTT prolonged), then it confirms the presence of an inhibitor
Heparin
Antiphospholipid antibodies
Coagulation factor specific inhibitors (often due to antibody development against specific factors)

51
Q

Fibrinogen

A

Normal reference range: 250-350 mg/dL
Increased fibrinogen increases chances of clotting and coronary artery/cerebrovascular events.
Also an acute phase reactant and rises with any condition that causes inflammation or tissue damage: cancer, inflammation/infection (RA, TB, pneumonia), glomerulonephritis, acute MI, stroke, pregnancy, eclampsia, etc.
Non-specific so not used as such
Decreased fibrinogen increases chances of hemorrhage after traumatic surgery.
Low level seen in DIC, liver disease, severe malnutrition, cancer

52
Q

D-Dimer

A

A fibrin degradation product made by plasmin cleaving the crossed-linked fibrin mesh
Normal range: < 250 ng/mL
Screen for:
DIC
Venous thrombosis (PE, DVT)
Acute MI
D-Dimer is more specific for DIC compared to other fibrin degradation products.

NCD final exam (2 decks)

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