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Peds II > Exam 1 - Hematology > Flashcards

Exam 1 - Hematology Flashcards

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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
What is G6PD deficiency?
Red cell enzyme defect that causes hemolytic anemia that causes episodic hemolysis
26
What causes episodic hemolysis in G6PD deficiency?
Oxidant stress of infection and certain drugs/foods
27
What is the clinical manifestations of G6PD deficiency?
Neonatal jaundice, hyperbilirubinemia, and episodic hemolysis
28
What will you see on peripheral smear with G6PD deficiency?
Heinz bodies
29
What will you see on peripheral smear with lead poisoning?
Basophilic stippling
30
What is the treatment for lead poisoning?
Chelation
31
What is the clinical presentation of congenital erythrocytosis (familial polycythemia)?
- Only RBCs are affected and hemoglobin may be as high as 27 - plethora and splenomegaly - HA and lethargy
32
What is the treatment for familial polycythemia?
Phlebotomy
33
What causes secondary polycythemia?
-occurs in response to hypoxemia (cyanotic congenital heart disease and chronic pulmonary disease)
34
What is the treatment for secondary polycythemia?
Correction of underlying disorder and phlebotomy
35
What is a normal platelet count? What platelet count has a risk of spontaneous bleeding?
Normal: 150,000-400,000 | Spontaneous bleeding: <20,000
36
What does PT measure?
The extrinsic and common pathways (VII and tissue factor)
37
What does PTT/ aPTT measure?
Intrinsic and common pathways (VIII, IX, XI, and XII)
38
What is INR used to measure?
A more acute reflection if PT, used to monitor warfarin treatment
39
What is bleeding time used to measure?
Time for hemostasis, a screen test for platelet dysfunction
40
What is the most common bleeding disorder of childhood?
Acute ITP
41
What is ITP?
An immune mediated attack against platelets that often follows an infection with viruses
42
What are the clinical manifestations of ITP?
Petechiae, ecchymosis, and epistaxis
43
What are the lab findings with ITP?
Thrombocytopenia, normal WBC, normal hemoglobin, and NORMAL PT and aPTT
44
What is the treatment of ITP?
-Avoidance of medications that compromise platelet function, bleeding precautions, prednisone, IVIG, and splenectomy
45
What is the most common inherited bleeding disorder?
Von Willebrand disease
46
What is Von willebrand disease? What are symptoms associated with it?
A decrease in the level of impairment in the action of Von willebrand factor (vWF) - Prolonged bleeding from mucosal surfaces - Easy bruising
47
What is vWF?
A protein that binds to factor VIII and is a cofactors for platelet adhesion to the endothelium
48
What lab is abnormal with Von Willebrand disease?
Prolonged bleeding time
49
What is the treatment for Von willebrand disease?
Desmopressin (causes release of vWF and factor VII from endothelial stores) VWF replacement therapy
50
What causes hemophilia A?
Factor VIII deficiency (most common)
51
What causes hemophilia B?
Factor IX deficiency (Christmas disease)
52
What is the clinical presentation of hemophilia?
Bleeding that can occur anywhere (common sites into joints and muscles)
53
What is hemarthrosis?
Bleeding into joints, result of severe hemophilia and can lead to joint destruction if recurrent
54
What lab is abnormal in hemaphilia?
Prolonged aPTT
55
What is the treatment of hemophilia?
- Desmopression (for hemophilia A) | - Factor replacement (VIII and IX) to achieve sufficient hemostasis
56
What is the Disseminated Intravascular coagulation (DIC)?
An acquired syndrome that results in hemorrhage and microvascular thrombosis that is triggered by either sepsis, trauma, or malignancy
57
What are the 3 acquired bleeding disorders?
DIC, Liver disease, and kidney disease
58
What are the symptoms of DIC?
Shock, diffuse bleeding tendency, and evidence of thrombotic lesions
59
What is seen on lab work in a patient with DIC?
- decreased platelet count - Prolonged aPTT and PT - decreased fibrinogen level - elevated D-diner and fibrinogen degradation products
60
What is the treatment of DIC?
- identification and treatment of the triggering event - replacement therapy for consumptive coagulopathy - Anticoagulant therapy when indicated
61
What are the vitamin K dependent factors?
II, VII, and IX, and X
62
What is the abnormal lab seen with vitamin K deficiency?
Prolonged PT and aPTT
63
What is treatment of vitamins K?
Treatment of underlying condition and vitamin K at birth
64
What does protein C do?
Inactivated Activated forms of factors V and VIII
65
What condition is warfarin-induced skin necrosis associated with?
Protein C deficiency
66
Protein S is a cofactors for ***.
Protein C
67
Patients with Factor V Leiden mutation have increased risk for what?
risk of VTE is increased two-to sevenfold
68
What is the clinical presentation of antithrombin deficiency?
VTE
69
The efficiency of heparin is significantly diminished in what condition?
Antithrombin deficiency
70
What is the most common type of vasculitis?
Henoch-schonlein purpura (HSP)
71
What is HSP?
Small vessel vasculitis caused by deposition of IgA immune complexes. -URI often precedes diagnosis
72
What is the clinical presentation of HSP?
- palpable purpura - arthritis and arthralgias - abdominal pain - renal disease
73
What are the lab findings of HSP?
- 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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