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(OMS2) SBL Exam 1 (Pathology + Clinical Medicine) > Red cell/White cell disorders, Leukemia/Lymphoma (Peds) > Flashcards

Red cell/White cell disorders, Leukemia/Lymphoma (Peds) Flashcards

1
Q

The physiologic nadir for hemoglobin in infants occurs at approximately?

A

2 months of age

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2
Q

What effect does anemia have on the hemoglobin-oxygen-dissociation curve?

This causes the affinity of hemoglobin for oxygen to become reduced in?

A

1) Concentration of 2,3-DPG increases within the RBC leading to the curve to shift to the right
2) Tissues needing to be oxygenated

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3
Q

At what rate of development for anemia does the body better compensate it?

What rate usually results in more dramatic symptoms?

A

1) Slowly developing anemia

2) Rapidly developing anemia

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4
Q

What does the MCV (mean corpuscular volume) tell you?

A

The mean volume of individual RBCs in a sample

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5
Q

What does the MCHC (mean corpuscular hemoglobin concentration) tell you?

A

Grams of HGB per 100 mL of RBCs

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6
Q

What does the RDW (Red blood cell distribution width) tell you?

A

Variability of RBC sizes in a sample

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7
Q

What does an H/H (hemoglobin/hematocrit) lab test tell you?

A

1) The amount of hemoglobin in the blood

2) The volume percentage of red blood cells in blood

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8
Q

What tells you if the RBCs are microcytic, normocytic, or macrocytic?

What tells you if the RBCs are hypochromic, normochromic, or hyperchromic?

A

1) MCV

2) MCHC

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9
Q

What are general causes of anemia?

A

1) Decreased RBC production (in bone marrow)

2) Increased RBC destruction

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10
Q

Hemoglobinuria, hemoglobinemia, and hemosiderinuria are due to what type of hemolysis?

A

Intravascular

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11
Q

Splenomegaly and hepatomegaly are due to what type of hemolysis?

A

Extravascular

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12
Q

Chronic blood loss will lead to what type of anemia?

A

Microcytic hypochromic anemia

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13
Q

A low number of reticulocytes in a patient with anemia is indicative of?

A

Inadequate bone marrow response

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14
Q

What is a normal bone marrow response to anemia?

A

Increased number of reticulocytes

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15
Q

What is a congenital pure red blood cell aplasia that almost always presents in infancy?

A

Diamond-Blackfan anemia

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16
Q

What does Diamond-Blackfan anemia result in?

A

Macrocytic anemia with low reticulocyte count

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17
Q

The most common inherited form of aplastic anemia is?

A

Fanconi anemia

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18
Q

What can fanconi anemia progress to?

We may not see symptoms until?

A

1) Pancytopenia

2) Around 10 years-old

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19
Q

Microcytic, hypochromic anemia in infants and toddlers is most often due to?

This is most likely caused by?

A

1) Iron deficiency

2) Dietary lack of iron

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20
Q

How could an peds patient present with iron deficiency anemia?

A

Pale and diet mostly made up of cow’s milk

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21
Q

Mentzer index is most helpful in distinguishing mild IDA from?

A

Beta thalassemia trait

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22
Q

If the Mentzer index is less than 13 it means?

If it’s greater than 13?

A

1) IDA

2) Beta thalassemia trait

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23
Q

How is absolute neutrophil count calculated?

A

ANC = ((%neutrophils + %bands) X (WBC)) / 100

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24
Q

Neutropenia occurs when a decrease in the absolute neutrophil count is less than?

Mild neutropenia: ANC = ?

Moderate neutropenia: ANC = ?
Severe neutropenia: ANC < ?

A

1) 1500/µL
2) 1000 - 1500
3) 500 – 1000
4) 500

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25
Q

What would lead poisoning show on a peripheral smear?

A

Basophilic stippling

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26
Q

If the ANC is < 500, the risk of what is high?

A

Serious infection

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27
Q

Impaired myeloid differentiation caused by maturational arrest of neutrophil precursors causes what condition?

A

Kostmann syndrome (Severe Congenital Neutropenia)

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28
Q

What are the clinical manifestations of Kostmann syndrome?

A

Life threatening pyogenic infections early in life

29
Q

What is the inheritance of Kostmann syndrome?

A

Autosomal recessive

30
Q

Kostmann syndrome increases the risk of?

A

AML

31
Q

Cyclic fever, oral ulcers, gingivitis, periodontal disease, and recurrent bacterial infections are clinical manifestation of what condition?

A

Cyclic neutropenia

32
Q

What is defected in cyclic neutropenia?

A

Stem cell regulatory defect

33
Q

What is the inheritance of cyclic neutropenia?

A

Sporadic or autosomal dominant

34
Q

What is the ANC for cyclic neutropenia?

A

ANC <200 for 3-7 days every 15-35 days

35
Q

Shwachman-Diamond syndrome is due to a triad of what?

A

1) Neutropenia
2) Exocrine pancreatic insufficiency
3) Skeletal abnormalities

36
Q

What is the inheritance of Shwachman-Diamond syndrome?

A

Autosomal recessive

37
Q

Shwachman-Diamond syndrome increases the risk for?

A

Myelodysplastic syndrome or leukemia

38
Q

What is defected in Shwachman-Diamond syndrome?

A

Neutrophil mobility

39
Q

Bone marrow failure syndrome with all cell lines affected results in?

A

Fanconi anemia

40
Q

What is the inheritance of Fanconi anemia?

A

Autosomal recessive

41
Q

Fanconi anemia increases the risk for?

A

AML, brain tumors, and Wilms tumor

42
Q

What does Leukocyte adhesion deficiency result in?

A
  1. Delayed separation of umbilical cord stump (>3 weeks)
  2. Recurrent and severe bacterial and fungal infections without pus accumulation
  3. Poor wound healing
43
Q

What is the inheritance of Leukocyte adhesion deficiency?

A

Autosomal recessive

44
Q

What is the cause of Leukocyte adhesion deficiency?

A

Neutrophils have diminished adhesion to surfaces so they can’t migrate out of blood vessels

45
Q

Hyperimmunoglobulin E syndrome (Job syndrome) causes a triad of?

A
  1. Severe eczema
  2. Recurrent bacterial infections (usually staph) of skin
  3. Recurrent pulmonary infections (bacterial, fungal)
46
Q

What is the inheritance of Hyperimmunoglobulin E syndrome (Job syndrome)?

A

Sporadic or autosomal dominant

47
Q

What is a very rare autosomal recessive disease that causes partial oculocutaneous albinism, neuropathies, and recurrent pyogenic infections?

A

Chediak-Higashi syndrome

48
Q

Chronic granulomatous disease is due to recurrent purulent infections with?

A

Fungal or bacterial catalase-positive organisms

49
Q

Chronic granulomatous disease is due to absent?

A

Superoxide

50
Q

What is the inheritance of Chronic granulomatous disease?

A

X-linked recessive

51
Q

What virus causes neutropenia and causes a slapped cheek appearance?

A

Parvovirus B19

52
Q

DiGeorge syndrome is associated with an absent?

This results in what type of immunodeficiency?

A

1) Thymus

2) T-cell immunodeficiency

53
Q

The #1 cause of death of children due to illness is?

A

Brain Cancer

54
Q

The most common malignancy between age 15-19 years-of-age is?

A

Hodgkin Lymphoma (HL)

55
Q

What infectious agent may be involved with Hodgkin lymphoma?

A

EBV

56
Q

What are considered a pathognomonic feature of HL?

A

Reed-Sternberg cells

57
Q

What are the B-signs used in the staging of Hodgkin lymphoma?

A

1) Unexplained fever
2) Weight loss
3) Night sweats

58
Q

Any patient with persistent, unexplained LAD unassociated with an obvious underlying inflammatory or infectious process should undergo?

Especially if?

A

1) CXR to rule out a mediastinal mass

2) They display respiratory symptoms

59
Q

What is the inheritance of Wiskott-Aldrich syndrome?

A

X-linked recessive

60
Q

What is the classic triad of Wiskott-Aldrich syndrome?

A

1) Recurrent sinopulmonary and ear infections
2) Severe atopic dermatitis
3) Bleeding secondary to significant thrombocytopenia

61
Q

Wiskott-Aldrich syndrome predisposes a patient to?

A

Non-Hodgkin lymphoma

62
Q

What condition commonly manifests as abdominal (sporadic type) or head and neck disease (endemic type) with involvement of the bone marrow or CNS?

A

Burkitt Lymphoma

63
Q

What account for the greatest percentage childhood malignancies?

A

Leukemias

64
Q

Trisomy 21 (Down Syndrome) is associated with what leukemias?

A

ALL and AML

65
Q

At what platelet count is clinically significant bleeding possible?

When is life threatening hemorrhage possible?

A

1) Less than 20,000 μl

2) Less than 10,000 μl

66
Q

The decreased platelet counts seen in Immune thrombocytopenic purpura is due to?

The platelets that are present have what distinct feature?

What precipitates ITP by about 1-2 weeks?

What do you treat it with?

A

1) Anti-platelet antibodies
2) Large
3) Viral illness
4) Prednisone

67
Q

What is Kasabach-Merritt phenomenon due to?

What is it associated with?

A

1) Thrombocytopenia and hypofibrinogenemia

2) Giant hemangioma and intravascular coagulation

68
Q

What are the signs and symptoms that are “red flags” in patients with thrombocytopenia?

A

1) Pancytopenia
2) Raised LDH (elevated in cancer)
3) Associated new renal impairment

69
Q

Hemolytic Uremic syndrome presents as classic triad of?

A

1) Microangiopathic hemolytic anemia
2) Thrombocytopenia
3) Renal damage

(OMS2) SBL Exam 1 (Pathology + Clinical Medicine) (16 decks)

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