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COMLEX STEP 1 2017 > HEME/ONC > Flashcards

HEME/ONC Flashcards

1
Q

What is Anisocytosis?

A

RBCs are of unequal size

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2
Q

What is Erythroblastosis fetalis?

A

maternal Ab attack and destroy fetal RBCs

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3
Q

What is Poikilocytosis?

A

RBCs are irregular in shape

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4
Q

What is Reticulocytosis?

A

prominence of immature RBCS (reticulocytes in peripheral blood)

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5
Q

What is Schistocytosis?

A

presence of hemolyzed or fragmented RBCs

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6
Q

What is observed in a patient with heparin-induced thrombocytopenia

A

DVT

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7
Q

What two things are low in the serum in iron deficiency anemia?

A
  1. serum iron

2. ferritin

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8
Q

Who does chronic lymphocytic leukemia most commonly affect? what are the Sx?

A

elderly

often asymptomatic peripheral lymphocytosis

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9
Q

What 2 blood Sx is Parvovirus B19 associated with (especially in patients with sickle cell disease)?

A

aplastic anemia

pancytopenia

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10
Q

What bone disease is Salmonella a common cause of in sickle cell patients?

A

Osteomyelitis

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11
Q

What translocation is most associated with Burkitt Lymphoma?

A

t(8, 14)

c-myc oncogene

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12
Q

What blood disorder do pts with G6PD deficiency tend to get after injesting foods/meds that induce oxidative stress?

A

hemolytic anemia

sulfonamids, primaquine, chloroquine, dapsone, isoniazid, and nitrofurantoin

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13
Q

Order the following types of Hodgkin lymphoma in descending order of commonality:

  1. Lymphocyte depleted
  2. Lymphocyte rich
  3. Mixed cellularity
  4. Nodular sclerosis
A
  1. Nodular Sclerosis
  2. Mixed Cellularity
  3. Lymphocyte depleted
  4. Lymphocyte rich
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14
Q

What is the triad of Sx for Hemolytic Uremic Syndrome (HUS)? What is the most common causing agent?

A
  1. Hemolytic anemia
  2. Acute Kidney injury
  3. Thrombocytopenia
    enterohemorrhagic E. coli
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15
Q

What enzyme catalyzes the rate limiting step in heme synthesis?

A

Aminolevulinic acid synthase (ALA synthase)

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16
Q

What is deficiency of ADAMTS13 associated with?

A

hereditary form of thrombotic thrombocytopenie purpura (TTP-HUS)

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17
Q

What is associated with defects in ankyrin and spectrin?

A

hereditary spherocytosis

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18
Q

What is associated with a defect in CD55 (decay accelerating factor)?

A

Paroxysmal nocturnal hemoglobinuria

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19
Q

What does Burkitt Lymphoma look like histologically?

common demographic and presentation?

A

starry sky appearance, sheets of lymphocytes with interspersed “tingible body” macrophages

A/w EBV
Jaw lesion in endemic form in Africa; pelvis or abdomen in sporadic form

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20
Q

What is the pathology of Immune thrombocytopenia (ITP)

A

immune mediated destruction of platelets from Abs directed against Gp IIb/IIIa

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21
Q

What is the microscopic indicator that most likely suggests Acute myeloid leukemia (AML)

A

Auer Rods

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22
Q 
What is the Dx?
Prolonged Bleeding time
Petechiae, purpura
menorrhagia
Decreased platelet count
presence of giant platelets, normal PT, PTT
A

Bernard-Soulier syndrome - deficiency of platelet glycoprotein Ib receptor

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23
Q

bone marrow/peripheral blood stains positive for tartrate resistant acid phosphatase (TRAP)

bone marrow biopsy - increased cells, monocytes with cytoplasmic projections

increased B-cells in late development*

A

Hairy Cell leukemia

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24
Q

How is Beta thalassemia diagnosed?

A 
Increased:
Hb A2 (MOST SPECIFIC)
Hb F (nonspecific)
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25
Q

How do you differentiate Multiple Myeloma and Waldenstroms macroglobulinemia

A

MM: IgG spike (not IgM), M PROTEIN spike

WM: -plasma cell infiltration into bone marrow

  • hyperviscosity syndrome (blurred vision, Raynaud phenomenon)
  • no “CRAB” findings

both have peripheral rouleaux

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26
Q

what does decreased serum haptoglobin suggest?

A

hemolytic process (haptoglobin binds free Hb)

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27
Q

Identify disease and tumor marker:
3-5 year old child
bleeding, bone pain, petechiae, fatigue/pallor
pancytopenia
bone marrow aspirate - increase immature cells of lymphoid lineage

A

Acute lymphoblastic leukemia (ALL)

  • stain positive for:
    1. terminal deoxynucleotidyl transferase (TdT)
    2. CALLA
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28
Q

What is the mechanism of septic shock activation by macrophage interaction with Bacterial Lipopolysaccharide?

A

Lipid A from Bacterial LPS binds to CD14 on Macrophages to initiated septic shock

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29
Q

Outline the location of fetal erythropoiesis?

A

Yolk sac: 3-8 weeks
Liver: 6 weeks-birth
Spleen: 10-28 weeks
Bone marrow: 18weeks - adult

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30
Q

How does Fetal hemoglobin (HbF) differ from adult hemoglobin (HbA1)

A 
HbF = alpha2gamma2
HbA1 = alpha2beta2
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31
Q

How does blood type AB and type O differ in ability to donate and receive PLASMA

A

AB: universal plasma DONOR (no anti-A, anti-B Ab in plasma)

O: universal plasma RECIPIENT

RBC donor and recipient is opposite

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32
Q

Which coagulation factors are inhibited by Warfarin

A

Warfarin = Vitamin K antagonist - interferes with gamma-carboxylation of Vit K dependent factors

II, VII, IX, X, C, S

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33
Q

What is the deficiency in the following:

  1. Hemophilia A
  2. Hemophilia B
  3. Hemophilia C
A
  1. VIII
  2. IX
  3. XI
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34
Q

Which factors are inhibited by antithrombin?

A

II, VII, IX, X, XI, XII

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35
Q

What other pathology is dermatomyositis associated with?

A

Dermatomyositis can present as a paraneoplastic disorder: ovarian, lung, colorectal, non-Hodgkin lymphoma

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36
Q

What gene defect is associated with Acute myelogenous leukemia (AML) - M3 variant (Acute promyelocytic leukemia)? What gene product is defective?

what is the presentation?

A

t(15;17) - translocation of PML gene on Chr 15 and RARA gene on Chr 17

= Abnormal Retinoic Acid Receptor = responds to all-trans retinoic acid
= anemia, thrombocytopenia, neutropenia, DIC

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37
Q

What does the lack of platelet aggregation in response to ristocetin most likely imply?

what is the inheritance pattern?

A

vonWillebrand Factor deficiency

Autosomal dominant

38
Q

G6PD deficiency displays what type of inheritance pattern?

A

X-linked recessive

39
Q

How does Folate (B6) deficiency differ from Cobalamin (B12) deficiency?

A

B6 deficiency - Increased Homocysteine, NO neuro sx

B12 deficiency, Increased Homocysteine + Methylmalonic acid, NEURO Sx

40
Q

How do Fanconi and Diamond-Blackfan anemia differ?

How are they similar?

A

Both show short stature, craniofacial abnormalities

Fanconi - pancytopenia
Diamond-Blackfan - only decreased RBC

41
Q

A pt with aggressive Non-hodgkin’s lymphoma have tumor cells that stain strongly for CD20. What monoclonal antibody would be most useful to add to her chemotherapy regiment?

A

Rituximab - specifically targets CD20

42
Q

What microorganism causes Hemolytic Uremic syndrome? what are abnormal lab values a/w this dz?

A

Shiga toxin producing organisms: E. coli O157:H7 or shigella dysenteriae

decreased hemoglobin, haptoglobin
increased serum lactate dehydrogenase
+ unconjugated bilirubin levels

43
Q

Follicular lymphoma is a/w with what chromosomal mutation and defective gene product?

A

t(14;18), Bcl-2 overexpression (anti-apoptotic fx)

44
Q

What electrolyte imbalances may occur after large volumes of blood transfusions

A

blood infusions are mixed with citrate anticoagulant
citrate can chelate calcium and magnesium

hypocalcemia, hypomagnesemia

45
Q

What two malignancies are Down Syndrome pts at a higher risk for?

A

Acute lymphoblastic leukemia (ALL)

Acute myelogenous leukemia (AML)

46
Q

What is the histological appearance of the bone marrow in idiopathic aplastic anemia?

A

pancytopenia with bone marrow hypocellularity

47
Q

Explain Bernard-Soulier syndrome

A

defect in platelet plug formation, large platelets

decreased GpIb - defect in platelet-vWF adhesion

48
Q

Explain Glanzmann thrombasthenia

A

defect in platelet plug formation

decreased GpIIb/IIIa - defect in platelet-platelet aggregation

lab: blood smear = no platelet clumping

49
Q

explain Thombotic Thombocytopenic purpura

etiology, labs, sx

A

Inhibition/deficiency of ADAMTS 13
-decreased degradation of vWF multimers
=increased platelet adhesion, aggregation, thrombosis
labs: schistocytes, increased LDH
sx: neuro, renal sx, fever, thrombocytopenia, microangiopathic hemolytic anemia

50
Q

What is the tx of vonWillebrand Disease?

A

Desmopressin - releases vWF from endothelium

51
Q

identify:

increased first of thrombotic skin necrosis with hemorrhage after administration of warfarin?

A

Protein C deficiency

52
Q

What are immune markers for Reed-Sternberg cells? what disease is this?

A

Hodgkin Lymphoma

CD15, CD30 Bcells

53
Q

What is the gene mutation w/e Mantle Cell lymphoma?

A

t(11;14) - translocation of cyclin D1 and heavy chain IgG

54
Q

What is the presentation of Mantle Cell lymphoma?

A

Adult males - very aggressive, typically present with late-stage disease

55
Q

What would be found on the peripheral smear?

middle aged man - progressive fatigue, weakness
abdominal discomfort, early satiety
Afebrile, Pallor, abdominal distention - Massive splenomegaly
Pancytopenia
No marrow can be aspirated

A

Lymphocytes with cytoplasmic projections - Hairy Cell leukemia

B-cell neoplasm
- bone marrow infiltration + cytokine production causes marrow fibrosis = pancytopenia
Massive splenomegaly from splenic red pulp infiltration

56
Q

What is the disease:
Acute onset abdominal pain, nausea, confusion
urine sample is reddish, darkens over 24 hours
intravenous dextrose improves Sx

A

Acute intermittent porphyria

accumulation of heme pathway intermediates (PBG, delta-ALA)
=acute GI, Neuro Sx

57
Q

Angiogenesis is driven by what two factors?

58
Q

What are Symptoms of Multiple Myeloma?

A 
"CRAB"
HyperCalcemia
Renal involvment
Anemia
Bone lytic lesions/Back pain

M Protein spike

59
Q

What physical finding can T cell Acute lymphoblastic Leukemia present as?

A

Mediastinal mass - SVC-like syndrome

60
Q

What chromosomal abnormality is a/w better prognosis of ALL

61
Q

What malignancy are smudge cells and mature lymphocytes seen in peripheral smear? relatively asymptomatic patients with unexplained elevated white blood cell count.

what is the most common type of malignant cells associated with this condition?

A

Chronic lymphocytic leukemia (CLL)

B-lymphocytes

62
Q

Identify:

  • Child
  • lytic bone lesions and skin rash or
  • recurrent otitis media with mass involving the mastoid bone
  • Birbeck granules - “tennis rackets”
A

Langerhans cell histiocytosis

63
Q

What mutation is a/w the chronic myeloproliferative disorders (Polycythemia vera, Essential thrombocythemia, Myelofibrosis)

A

JAK2 Mutations

64
Q

How does Nitrite toxicity manifest? Why?

A

Dusky coloration of skin (looks like cyanosis)

-Methemoglobin formation - leftward shift of oxygen curve (functional anemia)
partial pressure of O2 in blood is unchanged

65
Q 
identify dz and underlying cause:
pallor + fatigue
low erythrocyte count, Hb, reticulocyte percentage
normal WBC, platelet count
normal appearing bone marrow precursors
A

Pure Red Cell Aplasia (PRCA)

  • -inhibition of erythrocyte precursors by IgG autoAbs or cytotoxic T lymphocytes
  • -a/w Thymomas, lymphocytic leukemias, parvovirus B19
66
Q

What are the Hemologic findings of SLE?

A

Hemolytic anemia, thrombocytopenia, leukopenia

67
Q

What are renal findings of Paroxysmal nocturnal hemoglobinuria?

A

Hemosiderosis - from chronic hemolysis

–increases the risk of chronic kidney disease

68
Q

what is the mechanism of action of of heparin?

A

LMWH binds Antithrombin III which then binds factor Xa and stops factor Xa from converting prothrombin to thrombin

69
Q

Which three drugs can cause aplastic anemia?

A

Carbamazepine
Chloramphenicol
Sulfonamides

70
Q

Which bleeding disorder is clinically associated with Hemathrosis (bleeding in joints) - what is most likely deficient in this disorder?

A

Hemophilia - deficient factor VIII, IX

71
Q

What tumor marker is associated with CLL?

72
Q

Pt with irregularly irregular tachycardia is started on a med for stroke prevention. which med is this?

73
Q

18 month old boy presents with poor weight gain. used to be more active. Skull and facial bones are widened. pale skin, palpable hepatosplenomegaly. Radiolucent hyperplasia of the marrow along the edge of the skull.
Identify dz

A

Beta-thalassemia major - absent beta chains

newborns have HbF before transitioning to HbA. thats why symptoms appear 18 months after he was born

74
Q

What is the most common leukemia in:

  1. adults over the age of 30?
  2. children under the age of 15
A
  1. AML - stain w sudan black

2. ALL - do NOT stain w sudan black

75
Q

What pt with a PMH of a cerebrovascular accident (prescribed medication) presents with distinctive violaceous plaque, w areas of necrosis on her right upper extremity. Labs show decreased leukocytes, positive for gram-negative rods

what was the drug prescribed

A

Adenosine diphosphate receptor inhibitor

-antiplatelet drugs: Prasugrel, Ticlopidine, Clopidogrel
a/w neutropenia - can place pts at risk of developing severe infections and sepsis

76
Q

Pt recently treated for a bladder malignancy presents with constant ringing in his ears. exam shows bilateral high frequency hearing loss. this Sx is most likely the side effect of what drug? what is another side effect

A

Cisplatin - platinum based chemotherapy agent used to treat bladder cancer

  • adverse fx: NEUROTOXICITY (TINNITUS)
  • Platinum based agents are excreted by kidneys = NEPHROTOXICITY
77
Q

3 year old presents with recurrent abscesses, with increased episodes of epistaxis. Child is fair-skinned, blue-eyed, with metallic blonde hair. Irises have reduced pigmentation.

What is dz, inheritance pattern, and etiology?

A

Chediak-Higashi

  • -Autosomal Recessive
  • mutate LYST gene = defect in LYSOSOMAL STORAGE PROTEINS
  • -recurrent pyogenic infection, albinism
  • prog neuro problems
  • Coagulation defects
78
Q

43 yr old pt presents with gait ataxia, HA, dysdiadochokinesia, dysmetria on finger-nose testing. MRI shows present of cerebellar mass. CBC shows hematocrit 59%, hemoglobin 18.6

What is the mass, what is histology?

A

Hemangioblastoma
—paraneoplastic release of EPO!! = increased Hb, hematocrit

–foamy cells, increased vascularity

79
Q

What are physiological changes in carbon monoxide toxicity?

A

Left shift in Hb-O2 curve =

  1. decreased oxygen unloading in tissues
  2. decreased oxygen-binding capacity
  3. increased carboxyhemoglobin
80
Q

What is the deficient nutrient?
2 week old infant with conjunctival and facial pallor. born at 31 weeks via uncomplicated spontaneous vagina delivery. normal vital signs. Labs show neutropenia + hypochromic anemia

A

Copper deficiency - essential for hematopoiesis

deficiency = anemia, leukopenia

81
Q

What is deficient nutrient:
1 year old presents with ataxia, seizures. generalized hypotonia, hepatosplenomegaly, fragile silver-colored hair. microcytic hypochromic anemia, neutropenia

82
Q

What is the mechanism of lead poisoning on RBC’s? what are peripheral smear findings?

A

Basophilic stippling in the cytoplasm of RBCs

–Lead causes denaturation of enzyme RIBONUCLEASE = cant break down ribosomes, persistant ribosomes = basophilic stippling

inhibition of FERROCHELATASE

83
Q

14 year old presents with bleeding gums, Petechiae on trunk. Hx of flu-like symptoms last week. Decreased platelets, all other labs and peripheral smear are normal

what is disease?

A

Idiopathic thrombocytopenic purpura

need 2 fx for dx:

  1. isolated thrombocytopenia
  2. no external factors
84
Q

What drug is used to treat and prevent sickle cell crises in sickle cell anemia?

what is the mechanism of action?

A

Hydroxyurea - inhibits ribonucleotide reductase = increases HbF + decrease in sickling Hb

85
Q

what is the mechanism of paclitaxel (can be used in Kaposi sarcoma)

A

inhibits disassembly of microtubules

86
Q

What blood type is a/w gastric carcinoma

87
Q

What blood type is a/w peptic ulcers?

88
Q

23 yr old pregnant pt present with acute distress and tachycardia. BP is 90/60, HR 130. Petechial hemorrhages and blood oozing from mucous membranes. Labs show lower fibrinogen levels

  1. what is dz
  2. what are platelets, bleeding time, PT, PTT?
A
  1. Disseminated intravascular coagulation (DIC) - from presentation and low fibrinogen
  2. Low platelets, prolonged bleeding, PT, PTT
89
Q

What are lab findings for Anemia of Chronic dz? (MCV, Fe2+, TIBC)

A

normal MCV
normal Fe2+
low TIBC

90
Q

What is the mechanism of Etoposide?
What are side effects?
What is the use?

A

Topoisomerase II inhibitor = inhibits DNA synthesis (Act in S and G2 phases)

Myelosuppression, Gastrointestinal irritation, alopecia

Small cell lung Ca, Testicular carcinoma

91
Q

4 year old follows up after one weeks prior of mild viral infection. Physical exam reveals multiple bruises, petechiae, bleeding gums. Giant platelets are noted on peripheral blood smear and WBC, Hb are normal.

what other labs would be noted?

A

Acute Idiopathic thrombocytopenic purpura (ITP)
—-increase platelet destruction from anti GpIIb/IIIa Ab destruction

–increased megakaryocytes on bone marrow biopsy

92
Q

What is the Schilling test used to diagnose?

what would be a positive finding.

A 
Pernicious anemia
give:
1. oral radioactive cyanocobalamin (B12)
then,
2. non-radioactive B12 given intramuscularly

The IM B12 saturate B12 receptors so normal person excretes 7-22% of radioactive B12

if no intrinsic factor = decreased radioactive B12 urine levels

COMLEX STEP 1 2017 (19 decks)

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