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Peds II > Exam 1 - Hematology > Flashcards

Exam 1 - Hematology Flashcards

1
Q

What are the two kinds of anemia caused by bone marrow failure?

A

Fanconi anemia

Acquired aplastic anemia

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2
Q

What is fanconi anemia?

A

An inherited bone marrow failure syndrome that results from defective DNA repair.

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3
Q

What are the clinical manifestations of fanconi anemia?

A

Progressive Pancytopenia, congenital malformations, and increased incidence of malignancy

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4
Q

What is fanconi anemia often mistaken for?

A

ITP

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5
Q

What is the treatment for fanconi anemia?

A
  • supportive treatment for anemia, thrombocytpenia, and neutropenia
  • hematopoietic stem cell transplant
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6
Q

What are patients with fanconi anemia at high risk for?

A

Myelodysplastic Syndrome or Acute myeloid leukemia

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7
Q

What is acquired aplastic anemia?

A

Peripheral Pancytopenia with hypocellular bone marrow, 50% of childhood cases are idiopathic

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8
Q

What are the symptoms of aplastic anemia?

A

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

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9
Q

What complications can occur with aplastic anemia?

A

Overwhelming infection and severe hemorrhage

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10
Q

What is the most common nutritional deficiency in children?

A

Iron deficiency

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11
Q

When do children receive their first screening for anemia?

A

12 months

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12
Q

What will you see on blood work in IDA?

A

Microcytic, hypochromic anemia and ferritin <12mcg/L

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13
Q

What are the two kinds of megaloblastic anemia?

What are signs/symptoms associated with them?

A

Vitamin B12 and folic acid deficiency

Symptoms:

  • Pallor
  • Glossitis
  • Neuro symptoms (only in B-12)
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14
Q

What causes vitamin B12 deficiency?

A

Intestinal malabsorption and dietary insufficiency

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15
Q

Do neurologic symptoms occur with B12 or folate deficiency?

A

B-12

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16
Q

What disease is elevated methylmalonic acid seen with?

A

B-12 deficiency

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17
Q

What diseases is elevation of homocysteine seen with?

A

B-12 and folate deficiency

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18
Q

What is hereditary spherocytosis?

A

A red cell membrane defect that causes hemolytic anemia leading to jaundice, splenomegaly, and gallstones

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19
Q

What disease is spherocytes and increased osmotic fragility associated with?

A

Hereditary spherocytosis

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20
Q

What kind of anemia is thalassemia?

A

Microcytic, hypochromic anemia with normal RDW

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21
Q

What is used for diagnosis of thalassemia?

A

Hemoglobin electrophoresis

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22
Q

What is needed for diagnosis of sickle cell anemia?

A

Hemoglobin electrophoresis

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23
Q

What causes pain in sickle cell anemia?

A

Vaso-occlusion

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24
Q

What is the treatment for sickle cell anemia?

A

-Hydroyurea, treatment of painful occlusive episodes, stem cell transplant, and supportive

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25
Q

What is G6PD deficiency?

A

Red cell enzyme defect that causes hemolytic anemia that causes episodic hemolysis

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26
Q

What causes episodic hemolysis in G6PD deficiency?

A

Oxidant stress of infection and certain drugs/foods

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27
Q

What is the clinical manifestations of G6PD deficiency?

A

Neonatal jaundice, hyperbilirubinemia, and episodic hemolysis

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28
Q

What will you see on peripheral smear with G6PD deficiency?

A

Heinz bodies

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29
Q

What will you see on peripheral smear with lead poisoning?

A

Basophilic stippling

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30
Q

What is the treatment for lead poisoning?

A

Chelation

31
Q

What is the clinical presentation of congenital erythrocytosis (familial polycythemia)?

A
  • Only RBCs are affected and hemoglobin may be as high as 27
  • plethora and splenomegaly
  • HA and lethargy
32
Q

What is the treatment for familial polycythemia?

A

Phlebotomy

33
Q

What causes secondary polycythemia?

A

-occurs in response to hypoxemia (cyanotic congenital heart disease and chronic pulmonary disease)

34
Q

What is the treatment for secondary polycythemia?

A

Correction of underlying disorder and phlebotomy

35
Q

What is a normal platelet count? What platelet count has a risk of spontaneous bleeding?

A

Normal: 150,000-400,000

Spontaneous bleeding: <20,000

36
Q

What does PT measure?

A

The extrinsic and common pathways (VII and tissue factor)

37
Q

What does PTT/ aPTT measure?

A

Intrinsic and common pathways (VIII, IX, XI, and XII)

38
Q

What is INR used to measure?

A

A more acute reflection if PT, used to monitor warfarin treatment

39
Q

What is bleeding time used to measure?

A

Time for hemostasis, a screen test for platelet dysfunction

40
Q

What is the most common bleeding disorder of childhood?

A

Acute ITP

41
Q

What is ITP?

A

An immune mediated attack against platelets that often follows an infection with viruses

42
Q

What are the clinical manifestations of ITP?

A

Petechiae, ecchymosis, and epistaxis

43
Q

What are the lab findings with ITP?

A

Thrombocytopenia, normal WBC, normal hemoglobin, and NORMAL PT and aPTT

44
Q

What is the treatment of ITP?

A

-Avoidance of medications that compromise platelet function, bleeding precautions, prednisone, IVIG, and splenectomy

45
Q

What is the most common inherited bleeding disorder?

A

Von Willebrand disease

46
Q

What is Von willebrand disease?

What are symptoms associated with it?

A

A decrease in the level of impairment in the action of Von willebrand factor (vWF)

  • Prolonged bleeding from mucosal surfaces
  • Easy bruising
47
Q

What is vWF?

A

A protein that binds to factor VIII and is a cofactors for platelet adhesion to the endothelium

48
Q

What lab is abnormal with Von Willebrand disease?

A

Prolonged bleeding time

49
Q

What is the treatment for Von willebrand disease?

A

Desmopressin (causes release of vWF and factor VII from endothelial stores)
VWF replacement therapy

50
Q

What causes hemophilia A?

A

Factor VIII deficiency (most common)

51
Q

What causes hemophilia B?

A

Factor IX deficiency (Christmas disease)

52
Q

What is the clinical presentation of hemophilia?

A

Bleeding that can occur anywhere (common sites into joints and muscles)

53
Q

What is hemarthrosis?

A

Bleeding into joints, result of severe hemophilia and can lead to joint destruction if recurrent

54
Q

What lab is abnormal in hemaphilia?

A

Prolonged aPTT

55
Q

What is the treatment of hemophilia?

A
  • Desmopression (for hemophilia A)

- Factor replacement (VIII and IX) to achieve sufficient hemostasis

56
Q

What is the Disseminated Intravascular coagulation (DIC)?

A

An acquired syndrome that results in hemorrhage and microvascular thrombosis that is triggered by either sepsis, trauma, or malignancy

57
Q

What are the 3 acquired bleeding disorders?

A

DIC, Liver disease, and kidney disease

58
Q

What are the symptoms of DIC?

A

Shock, diffuse bleeding tendency, and evidence of thrombotic lesions

59
Q

What is seen on lab work in a patient with DIC?

A
  • decreased platelet count
  • Prolonged aPTT and PT
  • decreased fibrinogen level
  • elevated D-diner and fibrinogen degradation products
60
Q

What is the treatment of DIC?

A
  • identification and treatment of the triggering event
  • replacement therapy for consumptive coagulopathy
  • Anticoagulant therapy when indicated
61
Q

What are the vitamin K dependent factors?

A

II, VII, and IX, and X

62
Q

What is the abnormal lab seen with vitamin K deficiency?

A

Prolonged PT and aPTT

63
Q

What is treatment of vitamins K?

A

Treatment of underlying condition and vitamin K at birth

64
Q

What does protein C do?

A

Inactivated Activated forms of factors V and VIII

65
Q

What condition is warfarin-induced skin necrosis associated with?

A

Protein C deficiency

66
Q

Protein S is a cofactors for ***.

A

Protein C

67
Q

Patients with Factor V Leiden mutation have increased risk for what?

A

risk of VTE is increased two-to sevenfold

68
Q

What is the clinical presentation of antithrombin deficiency?

A

VTE

69
Q

The efficiency of heparin is significantly diminished in what condition?

A

Antithrombin deficiency

70
Q

What is the most common type of vasculitis?

A

Henoch-schonlein purpura (HSP)

71
Q

What is HSP?

A

Small vessel vasculitis caused by deposition of IgA immune complexes.
-URI often precedes diagnosis

72
Q

What is the clinical presentation of HSP?

A
  • palpable purpura
  • arthritis and arthralgias
  • abdominal pain
  • renal disease
73
Q

What are the lab findings of HSP?

A
  • Normal or elevated platelet count
  • Antistreptolysin O titer elevated
  • Serum IgA may be elevated
  • hemoccult may be positive

Peds II (11 decks)

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