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Hematologic Disorders Flashcards

1
Q

Definition of MTP

A

Replacement of approximately 10 units of pRBCs within 24 hours
Protocols may include early administration of FFP (2-3 units for every 3 units of pRBCs) and platelets (8-10 units or 1 apheretic unit for every 10u pRBCs)

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2
Q

What is the most common infection transmitted in blood transfusion?

A

Hepatitis B

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3
Q

Which hepatitis viruses are associated with development of HCC?

A

Hepatitis B and C

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4
Q

Most common presentation of SCD in infants

A

Dactylitis

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5
Q

Physiologic cause of muscle and cardiac manifestations in hyperkalemia

A
  • Impaired neuromuscular transmission after high extracellular potassium causes persistent depolarization
  • Causes inactivation of the sodium channels -> decreased membrane excitability
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6
Q

ECG changes due to hyperkalemia

A
  1. Peaked T wave
  2. Prolonged PR interval and dropped P wave
  3. Widened QRS complex
  4. Sine wave
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7
Q

ECG findings consistent with hyperkalemia are seen in the toxic manifestations of what drug?

A

Digoxin

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8
Q

When does heparin-induced thrombocytopenia usually occur?

A

Within 5-7 days of initiation of therapy

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9
Q

Tx for HIT

A

Direct thrombin inhibitor (lepirudin, argatroban)
Factor Xa inhibitor (fondaparinux)
Heparinoids (danaparoid)

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10
Q

4T score of HIT

A

Thrombocytopenia
Timing of platelet count fall
Thrombosis or other sequelae
Other cause for thrombocytopenia

Q#279895

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11
Q

Pathophysiology of transfusion-related acute lung injury

A

Acute neutrophilic response that leads to endothelial damage and massive capillary leak in the pulmonary vasculature

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12
Q

S/S of TRALI

A
  • Hypoxemia within 6 hrs of transfusion
  • Hypotension, tachycardia
  • Fever - not common in transfusion-associated circulatory overload
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13
Q

Medications useful in hemophilia associated bleeding

A

Severe Disease with deep laceration or muscle compartment bleeding -> factor VIII replacement
Severe Disease with superficial lac or abrasion -> topical thrombin
Mild Disease with minor mucosal or superficial bleeding -> desmopressin, aminocaproic acid or TXA

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14
Q

What agent provides the fastest reversal of warfarin-induced coagulopathy?

A

Prothrombin complex concentrate

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15
Q

What is the most serious transfusion reaction?

A

Hemolytic transfusion reaction

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16
Q

Cause of hemolytic transfusion reaction

A

Most commonly result of ABO incompatibility - alloantibodies formed in response to foreign red blood cell antigens.

Antibody-antigen complex reaction leads to intravascular destruction of transfused red blood cells

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17
Q

Symptoms of hemolytic transfusion reaction

A 
Immediate fever and chills
Headache
Nausea/Vomiting
Myalgias
Dark urine
Hypotension
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18
Q

Management of hemolytic transfusion reaction

A
  1. Stop the transfusion
  2. Immediate vigorous crystalloid infusion
  3. Diuretic therapy to maintain urine output 1-2 mL/kg/hr
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19
Q

What is the most common cause of hemolytic transfusion reaction due to ABO incompatibility?

A

Clerical error

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20
Q

Definition of neutropenic fever

A

Temperature of 38C for more than 1 hour or temperature of 38.3C once with an absolute neutrophil count less than 500/mm3

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21
Q

Calculating ANC

A 
ANC = WBC count x ((PMNs/100) + (Bands/100))
ANC = Total WBCs x %Total neutrophils (segmented + bands)
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22
Q

After chemotherapy, when is the expected nadir of the patient’s neutrophil count?

A

6-10 days

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23
Q

How long does it typically take the neutrophil count to recover after nadir?

A

5 days

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24
Q

Common malignancies that metastasize to bone

A
  • Breast
  • Lung
  • Thyroid
  • Kidney
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25
Q

Clinical features of multiple myeloma

A

CRAB - hypercalcemia, renal failure, anemia, lytic bone lesions

  • Bone pain - back and ribs
  • Spinal cord compression
  • Hypogammaglobulinemia (leading to sepsis)
  • Anemia
  • Hyperviscosity syndrome (Rouleaux formation)
  • M spike on serum protein electrophoresis
  • Bence-Jones proteins on urine analysis
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26
Q

Tumor lysis syndrome is most commonly seen after chemotherapy of what type of malignancies?

A

Hematologic including acute leukemias and high-grade non-Hodgkin lymphomas, particularly Burkitt lymphoma

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27
Q

Hallmarks of tumor lysis syndrome

A
  • Hyperkalemia from cytosol breakdown
  • Hyperphosphatemia from protein breakdown
  • Hypocalcemia 2/2 hyperphosphatemia
  • Hyperuricemia from DNA breakdown

Neuromuscular irritability, cardiac dysrhythmia, renal failure

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28
Q

Which type of lymphadenopathy in the neck area suggests underlying metastatic disease?

A

Supraclavicular lymphadenopathy suggests metastatic disease from chest or abdomen

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29
Q

What is the most common location for spontaneous bleeding in children with hemophilia A? What about adults?

A

Ankle (knee is most common joint in adults followed by elbow and ankle)

30
Q

What factor is deficient in hemophilia A?

A

Factor VIII (“A”ight)

31
Q

What factor is deficient in hemophilia B

32
Q

Lab values in iron deficiency anemia

A 
Iron <50 mcg/dL
Transferrin saturation percent <10%
Serum ferritin <15 mcg/L
Total Iron Binding Capacity >400 mcg/dL
Decreased reticulocyte counts
33
Q

Causes of acquired sideroblastic anemia?

A
  • Lead toxicity
  • Pyridoxine deficiency
  • Drug toxicity (chloramphenicol, isoniazid)
  • Carcinoma
  • Leukemia
  • Chronic alcohol use
  • Infection
34
Q

Lab values in DIC

A

HIGH: PT, aPTT, thrombin clotting time, d-dimer, fibrin complexes

LOW: platelets, fibrinogen

35
Q

Causes of DIC

A
  • Obstetrical catastrophes such as amniotic fluid embolism or placental abruption
  • Metastatic malignancy
  • Massive trauma
  • Sepsis
  • Bacterial endotoxin activation
  • Snake envenomation
36
Q

Tx of DIC

A

Treat underlying condition

If bleeding complications -> replete clotting factors and platelets with FFP and platelets

37
Q

Examples of medications that can cause thrombocytopenia

A
  • Heparin
  • Certain antibiotics
  • Ranitidine
  • Phenytoin
38
Q

What happens to PT/PTT in patients with primary immune thrombocytopenia?

A

Remains normal

39
Q

Tx for ITP

A

IVIG, Steroids, possibly anti-D antibody

40
Q

Clinical features of polycythemia vera

A
  • Fatigue
  • Pruritus (especially following warm bath or shower)
  • Facial plethora (ruddy cyanosis)
  • Erythromelalgia (burning pain and erythema, pallor, or cyanosis)
  • Engorged retinal veins
  • Thrombosis
  • Gouty arthritis
  • Splenomegaly
41
Q

Tx for polycythemia vera

A
  • Hydroxyurea
  • Phlebotomy
  • Aspirin
42
Q

Mutation found in polycythemia vera

A

JAK2 mutation - chronic myeloproliferative neoplasm with abnormal proliferation in all three cell lines

43
Q

Which gastric hormone is hypersecreted, resulting in PUD and pruritus, common complications of polycythemia vera?

44
Q

Characteristics of CML (chronic myelogenous leukemia)

A
  • WBC elevated, but differential is fairly normal
  • Basophilia on smear
  • Hallmark = Philadelphia chromosome - reciprocal translocation of material b/n chromosomes 9 and 22
  • Triphasic:
    1. Chronic phase - fatigue, weight loss, diaphoresis, abdominal fullness and hepatosplenomegaly
    2. Accelerated phase - bleeding and petechiae
    3. Blast phase - greater than 20% blasts and resembles acute leukemias
45
Q

Characteristics of ALL (acute lymphocytic leukemia)

A
  • Most common childhood leukemia

- 75% affects B-cell precursors, 20% affects T-cell precursors

46
Q

Characteristics of CLL (chronic lymphocytic leukemia)

A
  • Most common adult chronic leukemia

- Smudge cells

47
Q

Characteristics of AML (acute myelogenous leukemia)

A
  • More common in adults than children

- Auer rods

48
Q

What is a risk factor for development of CML?

A

Exposure to ionizing radiation

49
Q

Tx for CML

A

Allogenic HSCT

Imatinib

50
Q

What is a Heinz body?

A
  • Inclusion within RBC composed of denatured hemoglobin
  • Formed as a result of oxidative damage
  • Macrophages in spleen remove the denatured hemoglobin which results in bite cells

Seen in G6PD deficiency

51
Q

What type of hereditary pattern for G6PD?

A

X-linked recessive

52
Q

A way to tell Systemic (B) symptoms of lymphoma from malaria

A

Malaria does not have lymphadenopathy

53
Q

Pathophysiology of TTP

A

Inhibition of enzyme ADAMTS13 which leads to large multimers of von Willebrand factor

54
Q

Tx of TTP

A

Plasmapheresis and consider corticosteroids for inadequate response to plasmapheresis and splenectomy for recurrent cases

55
Q

What is the most common cause of vitamin B12 deficiency?

A

Chronic malabsorption, as seen in pernicious anemia

56
Q

Complications of massive transfusion

A
  • Hypocalcemia
  • Metabolic acidosis
  • Hyper/hypokalemia
  • Hypothermia
  • Dilutional coagulopathy
  • Thrombocytopenia
  • ARDS
57
Q

When should calcium gluconate be given for hypocalcemia from massive transfusion?

A

Only if there are ECG changes

58
Q

Tx for blast crisis in patients with leukemia

A

Aggressive fluid resuscitation or phlebotomy and replacement with IV fluids

In patients who may not be able to tolerate large volumes of fluid -> leukapheresis

59
Q

Which tx for hyperkalemia should be avoided in treating tumor lysis syndrome?

A

Rectal sodium polystyrene sulfonate

60
Q

What is the most common risk factor for new-onset childhood immune thrombocytopenia?

A

Viral infection

61
Q

Tx for polycythemia vera

A

Low dose aspirin
Phlebotomy to a HCT of 45%
If with hyperviscosity syndrome - require IV fluid hydration and hematology consultation

Other options include hydroxyurea, interferon-alpha, ruxolitinib (JAK1 and JAK2 inhibitor)

62
Q

Mechanism of action of rivaroxaban, apixaban and edoxaban

A

Direct factor Xa inhibitor

63
Q

Intrinsic and extrinsic pathways

64
Q

What bacteria are most commonly implicated in acute chest crisis?

A

Chlamydia pneumoniae in adults and Mycoplasma pneumoniae in children

65
Q

What is Waddell triad?

A

A femur fracture, abdominal or pelvic injury, and head injury in school-age children hit by a car

66
Q

Hemolysis labs

A

Decreased haptoglobin
Increased LDH
Increased indirect bilirubin

67
Q

Symptoms of hemolytic transfusion reaction

A

Immediate fever and chills

  • headache
  • n/v
  • myalgias
  • dark urine
  • hypotension
  • back pain
  • shortness of breath
68
Q

Management of hemolytic transfusion reaction?

A
  1. Stop transfusion
  2. Immediate vigorous crystalloid infusion
  3. Diuretic therapy to maintain urine output 1-2mL/kg/hr
69
Q

Mechanism of hemolytic transfusion reaction

A

Host antibody attack of donor cells

70
Q

What blood product is associated with highest risk of febrile nonhemolytic transfusion reaction?

A

Platelets (approximately 5% incidence)

71
Q

Labs in von Willebrand Disease

A

Normal platelets
Normal PT/INR
Normal aPTT (prolonged if very low factor VIII)
Decreased factor VIII and prolonged bleeding time

Rosh Review (22 decks)

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