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PANCE Prep- Hematology Flashcards

1
Q

___ presents in adults with a cell lineage of the immature granulocytic cells seen in the peripheral blood e.g. myeloblasts, promyelocytes.
-Auer rods also are commonly seen in this condition

A

Acute myelogenous leukemia (AML)

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2
Q

When a patient receives more than one total blood volume (10 units) without replacement of ___, the patient will continue to bleed without administration of ___

A

clotting factors

fresh frozen plasma

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3
Q

A positive direct Coombs’ test may be seen in what condition

A

Autoimmune hemolytic anemia

hereditary spherocytosis has negative coombs

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4
Q

Hereditary spherocytosis is detected utilizing ___

A
  1. the osmotic fragility test

2. negative Cooombs

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5
Q

___ is indicated in Coumadin overdosage

A

Fresh frozen plasma

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6
Q

___ is indicated in type 2 and 3 patients with von Willebrand disease bleeding

___ is indicated in type 1 von Willebrand disease bleeding

A

vWF concentrate

DDAVP** (vasopressin) used to bleeding

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7
Q

__ is indicated for patients with Hemophilia A.

A

Factor VIII or cryoprecipitate

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8
Q

Once the diagnosis of heparin induced thrombocytopenia is suspected treatment should include:

A
  1. immediate discontinuation of all forms of heparin

2. treatment with a direct thrombin inhibitor should begin

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9
Q

___ is used for heparin overdosage

A

Protamine sulfate

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10
Q

What condition is caused by a disorder in the red blood cell membrane?

A

Hereditary spherocytosis

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11
Q

What Rh genotype of the mother and fetus puts the baby at risk for hemolytic disease of the newborn?

A

Mother: Rh negative
Fetus: Rh positive

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12
Q

Low molecular weight heparin (LMWH) dosage is based on which of the following?

A

Weight

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13
Q

___ is the diagnostic study of choice for G6PD deficiency.

A

G6PD assay

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14
Q

___ is indicated in the evaluation of hemolytic anemia

A

Hemoglobin electrophoresis

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15
Q

An African American male is placed on hydroxychlorquine (Plaquenil) for travel to Africa. Within six weeks he presents complaining of fatigue. CBC shows anemia. Which of the following is the diagnostic study of choice in this patient?

A

G6PD assay

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16
Q

CML (chronic myeloid leukemia) patients typically present with what physical exam finding

A

splenomegaly

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17
Q

Hereditary spherocytosis presents with what type of anemia

A

a normocytic, normochromic anemia and many spherocytes.

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18
Q

___ presents with a prolonged aPTT and normal platelet count and function.

A

Hemophilia A

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19
Q

An 18 year-old woman presents to the clinic complaining of fatigue. She reports a past history of lifelong frequent nosebleeds and bleeding gums. She also has menorrhagia. Her mother and maternal grandfather have a similar bleeding history. Initial lab results are as follows: WBC 9,500/mm3, Hgb 10.9 g/dL, HCT 33%, MCV 69 fL, MCHC 26 pg and platelets 284,000/mm3. Which of the following tests should be ordered to evaluate this patient’s diagnosis?

A

Bleeding time and platelet aggregometry

*presentation is consistent with a congential qualitative platelet disorder, most likely von Willebrand’s Disease, necessitating a bleeding time and evaluation of platelet function.

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20
Q

What therapies is recommended for a 13 month-old child with sickle cell disease?

A

Folic acid and penicillin V

*Ferrous sulfate is not globally recommended for patients with sickle cell disease.

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21
Q

What Leukemia is most closely associated with the Philadelphia chromosome?

A

chronic myelogenous leukemia

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22
Q

___ is used to treat immune-mediated hemolytic anemias

A

Prednisone

Splenectomy- long term definitive tx

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23
Q

immune-mediated hemolytic anemias presents with ___ red blood cells.

A

normocytic, normochromic

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24
Q

___ is characterized by bleeding from many sites as all coagulation factors are consumed and then broken down, leading to:

  1. decreased fibrinogen level and platelet count,
  2. prolonged PT and PTT
  3. presence of FIBRIN SPLIT products.
A

Disseminated intravascular coagulation

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25
Q

____ transports Fe throughout the body. This carrier can be measured indirectly by ____

A

transferrin

TIBC

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26
Q

Describe transferrin, TIBC and ferritin levels in iron deficiency anemia and anemia of chronic disease

A

Fe deficiency- Increased transferrin, increased TIBC, decreased ferritin

anemia of chronic disease- Decreased transferrin, decreased TIBC, and increased ferritin

*both have decreased serum Fe

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27
Q

HgbS on hemoglobin electrophoresis

A

sickle cell anemia (hemolytic)

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28
Q

Microcytic anemia with normal/increased serum Fe or no response to Fe tx

A

Thalassemia

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29
Q

How to differentiate alpha and beta thalassemia

A

alpha: hemoglobin electrophoresis: normal Hgb ratios of HbgA, A2, and F
beta: hemoglobin electrophoresis: DECREASED HgbA, INCREASED HgbF, increased HgbA2,

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30
Q

G6PD is associated with what?

A
  1. sulfa drugs (plaqunil, UTI meds- Bactrim)
  2. fava beans
  3. infections
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31
Q

TTP Pentad

A
  1. thrombocytopenia
  2. hemolytic anemia
  3. kidney damage
  4. Neurologic sx*
  5. Fever*
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32
Q

HUS Triad

A
  1. thrombocytopenia
  2. hemolytic anemia
  3. kidney damage* (more so than TTP)
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33
Q
  • Rouleaux Formation
  • RBCs stick together like “stack of coins”
  • Increased ESR
A
  • high protein
  • Multiple Myeloma
  • Infections
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34
Q

Bite cells (degmacytes) are seen with:

A

thalassemia, G6PD

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35
Q

Schistocytes (fragmented RBCS) are seen w/

A

Hemolytic anemias (DIC, TTP, HUS)

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36
Q

Keratocytes (helmet shaped RBCs) are seen w/

A

TTP, HUS, DIC, prosthetic valves

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37
Q

Echinocytes “Burr cells” are seen w/

A

Uremia

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38
Q

Hypersegmented neutrophils are seen w/

A

B12** and folate deficiencies

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39
Q

Presentation:

  • *neurological sx, paresthesias (stocking and glove-like paresthesia), gait abnormalities, memory loss, dementia (acute or permanent)
  • diarrhea, glossitis, anorexia
  • macrocytic anemia
A

B12 (cobalamin) deficiency

TX: IM B12- watch for signs of HYPOKALEMIA

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40
Q

How do you dx B12 and folate deficiency

A
  • Peripheral smear: MCV >115, hypersegmented neutrophils
  • increased homocysteine, increased methylmalonic acid*
    • Schilling test

*normal methymalonic acid with folate def.

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41
Q

Causes of macrocytic anemia

A
  1. B12 (cobalamin) def.
  2. Folate def.
  3. ETOH abuse
  4. liver disease
  5. hypothyroidism
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42
Q

Causes of microcytic anemia

A
  1. iron def.
  2. alpha/beta thalassemia
  3. early anemia of chronic disease (ACD)
  4. lead poisoning
  5. sideroblastic anemia
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43
Q

Presentation:

  • pagophagia, pica, angular cheilitis, koilonychia
  • microcytic, hypochromic anemia
A

Iron def. anemia

TX: ferrous sulfate 325mg orally daily

  • Vit. C increases Fe absorption
  • see increase in reticulocyte count w/in 7 days
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44
Q

dysphagia + esophageal webs + atrophic glossitiis + Fe def.

A

Plummer-Vinson syndrome

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45
Q

How do you dx Fe def. anemia

A

Decreased ferritin, increased TIBC, decreased serum FE

  • Increased RDW*,
  • decreased transferrin saturation <15%
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46
Q

Presentation:
abdominal pain w/ constipation, neurologic symptoms (ataxia, fatigue, learning disabilities, coma, shock, metabolic acidosis
-basophilic stippling and ringed sideroblasts

A

lead poisoning anemia (plumbism)

47
Q

How do you dx lead poisoning anemia (plumbism)

A

Peripheral smear: microcytic, hypochromic anemia w/ BASOPHILIC STIPPLING and RINGED SIDEROBLASTS

  • Increased serum lead and serum Fe,
  • Xray: “lead lines”
48
Q

Normally after ___ old, adult Hgb is predominant Hgb produced

A

6 months (HgbA- 2 alpha and 2 betas)

49
Q

Think ___ if microcytic anemia w/ normal/increased serum Fe or no response to Fe tx

A

Thalassemia

50
Q

How do you dx thalassemia

A
  • hypochromic, microcytic anemia w/ normal Fe
  • Perpherial smear: TARGET cells, teardrop cells
  • HEINZ bodies in HgbH disease
  • Hgb Electrophoresis**
51
Q

G6PD affects most commonly in

A

primarily AA males

52
Q

how do you dx sickle cell disease

A

decreased Hgb, HCT and increased reticuolocytes
-sickled erythrocytes +/- Howell-Jolly bodies**
Hgb electrophoresis*

53
Q

Tx of sickle cell disease

A
  1. pain control: IV hydration and oxygen in pain crisis
  2. Hydroxyurea: reduces freq. of pain crises
  3. Folic acid
  4. immunize for S. pneumococcus, H. influena and N. meningoccus *SHiN)
54
Q

Common presentations of sickle cell disease

A
  1. Dactylitits MC 1st presentation at 6-9 months
  2. Osteomyelitis (esp. Salmonella)
  3. Functional asplenia
55
Q

Dark cola-colored urine during the nigh tor early AM w/ partial clearing during the day
-thrombosis ex. hepatic, mesenteric

A

paroxysmal nocturnal hemoglobinuria

DX: flow cytometry best screening test*

TX: Eculizumab

56
Q

Primary hemostasis involves __

Secondary hemostasis involves __

A

Primary hemostasis- platelets form a plug at site of vascular injury: platelet adhesion activation and aggregation

Secondary hemostasis- clotting factors respond in a cascade to form fibrin strands which strengthens the platelet plug

57
Q

What does PT and PTT measure and give examples of things that prolong them

A

PT: EXTRINSIC factor pathway: (7 and 10**)
ex. heparin, DIC< vWD, hemophilia A and B

PTT: INTRINSIC: (8, 9, 11, 12**)
ex. warfarin, Vit K def, DIC

58
Q

heparin OD antidote:

warfarin OD antidote:

A

heparin: protamine sulfate
warfarin: vit K

59
Q

Decreased AdamTS12

A

primary TTP

tx: plasmapheresis

60
Q

Seen predominately in children (usually preceded by E. coli O157:H7, shigella or salmonella gastroenteritis:
-thrombocytopenia, microangiopathic hemolytic anemia, kidney failure (uremia)

A

HUS (hemolytic uremic syndrome)

TX: observation / IV fluids to maintain renal perfusion

  • plasmapheresis if severe
  • *Antibiotics worsen the condition
61
Q

Etiologies of DIC

A
  1. infections (gram neg. sepsis MC)
  2. malignanices
  3. Obstetrics
  4. massive tissue injury
62
Q

how do you DX DIC

A
  1. Increased thrombin, decreased fibrinogen, increased PTT/PT/INR,
  2. increased d-dimer (increased fibrinolysis)
63
Q

TX: DIC

A

tx underlying cause

2. FFP if severe bleeding

64
Q
  • BIMODAL peaks at 20 and again at 50y/o
  • Associated with EBV- MC in males
  • PAINLESS lymphadenopathy but may develop painful lymph nodes w/ ALCOHOL
A

Hodgkin Lymphoma

DX: biposy: REED-STERNBERG cell pathognomoic**
mediastinal lymphadenopathy

TX: radiation +/- chemo

**highly curable compared to NHL

65
Q
  • PAINLESS lymphadenopathy peripheral MC- extranodal sites common: GI, skin, CNS MC
  • abdominal pain
A

Non-hodgkin lymphoma

tx: unpredictable course
- follicular: Rituximab: ab vs CD20 on B cells
- Diffuse large B cell: chemo

66
Q

Describe presentation of multiple myeloma

A

“Bones BREAK” or CRAB
-(Ca elevated, Renal failure, Anemia, Bone osteolytic lesions)

Bone pain: osteolytic, destructive lesion
Recurrent infections from leukopenia
Elevated Calcium (hypercalcemia)
Anemia
Kidney failure (Bence Jones proteins in urine)

67
Q

DX and tx of multiple myeloma

A

DX:

  • serum protein electrophoresis: monoclonal (M) protein spike
  • urine protein electrophoresis: Bence-Jones proteins*
  • CBC: ROULEAUX formation and increased ESR
  • Skull radiograph: punched-out lytic lesions
  • **BM biopsy: plasmacytosis <10%

TX: autologous stem cell transplant is definitive tx +/- chemo

68
Q

MC childhood malignancy and common with Downs syndrome
Fever MC sx
CNS sx, hepatosplenomegaly, lymphadenopathy

A

ALL

DX: BM: hypercellular w/ >20% blasts

TX: combo chemo

69
Q

MC leukemia in adults

  • fatigue, painful lymphadenopathy
  • SMUDGE cells on peripheral smear
A

CLL (B cell)

TX: observe if indolent, Fludarabine chemo if progressive

70
Q

lab abnormalities and tx of tumor lysis syndrome

A

hyperuricemia, hyperkalemia, hypocalcemia, hyper phosphatemia, acute renal failure

allopurinol, IV fluid

71
Q

What leukemia is associated with Philadelphia chromosome

A

CML (Philadelphia CreaM cheese)

72
Q

Overproduction of all 3 myeloid cell lines- lymphocyte line normal

  • JAK2 mutation
  • hypervisocity or thrombosis
  • Splenomegaly,
A

Polycythemia vera (primary erythrocytosis)

73
Q

increased iron, ferritin, and transferrin

  • liver dysfunction
  • HF, cardiomyopathy, arrhythmias
  • hypogonadism
  • pancreatic insufficiency
  • Metallic or Bronze skin– bronze diabetes
A 
Hereditary hemochromatosis 
(excessive iron deposition in heart, liver, pancreas and endocrine organs)

DX: liver biopsy is gold standard– hemosiderin

tx: phlebotomy

74
Q

MC inherited caused of hypercoagulability

A

Factor V leiden

75
Q

MC clotting disorders

A

Factor V leiden
Protein C def.
Antithrombin III Def.

76
Q

Pernicious anemia is due to deficiency in what

A

intrinsic factor (secreted by parietal cells)

77
Q

MC cause of hypercalcemia in malignancy

A

Increased production of parathyroid hormone-related peptide (PTHrP)

*MC CA- lung, breast CA and myeloma

2nd MC cause is osteolytic metastasis (NOT OSTEOBLASTIC)

78
Q

PMH: miscariages

presents w/ clot, lacy erthematous rash on extremities

A

Antiphosopholipid antibody syndrome

DX: anticardiolipid antibodies, lupus anticoagulant, and anti-beta 2 glycoproteins

79
Q

Presentation:

  • 70y/o pt.
  • CBC: leukocytosis and lymphocytosis (usually found incidentally on ROUTINE bloodwork)
  • 1/3 develop autoimmune hemolytic anemia, and 5% develop ITP
A

Chronic lymphocytic leukemia (B Cell)

*presents with a WBC count greater than 20,000/microliter and absolute lymphocyte count of greater than 5000/microliter

80
Q

Spontaneous hemarthroses are pathognomonic for:

A

Hemophilia A and B

81
Q

What is the most serious delayed toxicity that is seen w/ the anthracycline drug class?

A

cardiomyopathy

82
Q

Bleomycin used in Hodgkin’s lymphoma and testicular CA has the known toxicity of ___

A

Pulmonary toxicity

83
Q

Common findings and tx of Primary polycythemia

A
  1. Elevated Hgb and HCT
  2. Elevated platelet- thrombosis/hypervisocity
  3. Splenomegaly
  4. facial plethora (flushed face*)
  5. JAK2 mutation
  6. engorged retinal veins

TX: phlebotomy

(diuretic use is suggestive of spurious PV)

84
Q

4 main common types of myeloproliferative neoplasms

A
  1. polycythemia vera (PV)
  2. essential thromboytosis
  3. CML
  4. primary idiopathic myelofibrosis
85
Q

Presentation:

  • Bleeding gums, epistaxis
  • NO splenomegaly
  • Hx of URI
  • Purpura or petechiae
  • Low platelets
A

Immune thrombocytopenia purpura (ITP)

TX: steroids***** +/- IVIG
*transfusion or splenectomy if platelet count drops below 5,000-10,000

TX in Adults: steroids or Rituxan and Eltrombopag are approved for ADULTS ONLY

86
Q

If left untreated, Vit. B12 def. can cause

A

Irreversible neuropathy

87
Q

Vit. B12 is produced by ___ and absorbed in ___

A

parietal cells

terminal ileum

88
Q
  • Decreased WBC, RBCS and platelets (pancytopenia)
  • BM biopsy: hypoplastic marrow, NO blasts
  • Causes: radiation exposure, chemical exposure to pesticides or solvents, viruses, paroxysmal nocturnal hemoglobinuria and sulfa compounds
A

aplastic anemia

*if farmer patient think aplastic anemia

89
Q

How do you dx Von Willebrand’s Disease

A
  1. S/S: Mucocutaneous bleeding: easy bruising, epistaxis, gums GI, menorrhagia
  2. decreased vWF levels
  3. +/- prolonged PTT
    * ** PTT prolongation worsen w/ aspirin
  4. Decreased ristocetin activity test gold standard**

MC bleeding disorder- inherited autosomal dominant

TX: Vasopressin or DDAVP

90
Q

Presentation:

platelet count starts to drop after being on coumadin and heparin and start developing thromboses

A

Heparin-induced thrombocytopenia (HIT)

*caused by autoantibodies of platelet factor 4 (PF4)

91
Q

Myelodysplastic Syndrome can progress to ___ in select patients

A

acute leukemia

92
Q

Packed red cell transfusions (PRBCs) can cause temporary ___

Risk of iron overload has been recognized after packed red blood cell transfusion of __ units

A

HYPERkalemia

more than 20 units

93
Q

__ products carry the highest risk of tranfusion-related acute lung injury (TRALI) among all blood products

A

plasma, apheresis platelets, and whole blood

TX: oxygen and possibly ventilation

*occurs w/in 6 hours of transfusion (presentation: hypoxia, pulmonary infiltrates, hypotension, cyanosis)

94
Q

One unit of Packed red cell transfusions (PRBCs) is expected to raise the hemoglobin ___

A

1 Hgb

95
Q

The following peripheral smears match what diseases?

  1. Target Cells
  2. Howell-Jolly bodies
  3. Heinz Bodies
  4. Rouleaux formation
  5. Poikilocytosis
  6. Bence Jones protein in urine
A
  1. Target Cells: liver dz, hemoglobinopathy like thalassemia
  2. Howell-Jolly bodies: post-splenectomy, splenic dysfunction, sickle cell
  3. Heinz Bodies: G6PD
  4. Rouleaux formation (stacked coin appearance of RBCS): myeloma
  5. Poikilocytosis: extreme iron deficiency
  6. Multiple myeloma
96
Q

Presentation:

  • Hx of RA
  • Splenomegaly and neutropenia
A

Felty’s syndrome

DX: Clinical, CBC, +/- BM biopsy to r/o other causes

97
Q
  • Prolonged PTT

- Mixing study of blood to normal serum results in persistently prolonged PTT

A
  • lupus anticoagulant
  • Factor 8 inhibitors
  • Contamination w/ heparin
  • PTT stays prolonged= presence of an inhibitor
  • if PTT corrects w/ mixing study= deficiency of one of the factors (12, 11, 9, 8)
98
Q

What med is the best choice in patients on chemotherapy to help minimize the risk of neutropenia

A

Pegfilgrastim (long acting colonystimulating factory (CSF) support)

99
Q

Sickle cell disease (HgbS) is what type of inherited disease?

A

autosomal dominant

*genetic counseling and testing should be performed if fhx even if healthy could have sickle cell trait

100
Q

Most common etiologies of Iron deficiency anemia

A
  1. CHRONIC blood loss (not acute)

2. Pregnancy- bc of increased production of erythrocytes

101
Q

When using tamoxifen in the treatment of malignancy, one must keep what toxicity in mind?

A

secondary malignancy- increased risk of uterine cancer

102
Q

What tumor marker can be used in the screening of patients for cancer?

A

alpha-fetoprotein- used to screen for hepatocellular carcinoma
(screen in those w/ chronic hep B, C and cirrhosis)

*PSA, CEA, CA 19-9 are not sensitive or recommended

103
Q

What is Christmas disease

A 
Hemophilia B (factor 4)
-X-linked recessive disease affecting primarily males
104
Q

Vitamin K deficiency occurs primarily with ___

A

primary biliary cirrhosis

105
Q

what is the most common cause of hypercalcemia in malignancy?

A

osteoblastic lesions from bone metastasis

106
Q

does Hodgkin’s lymphoma or non-Hodgkin’s lymphoma have Reed-Sternberg cells

A

Hodgkin’s

107
Q

Hodgkin’s lymphoma has what type of age distribution?

There is also an increased incidence of it after exposure to ___

A

bimodal

EBV

108
Q

Evaluation of ___ can suggest myelodysplastic syndrome (MDS) but ___ is diagnostic

A

peripheral smear

BM biopsy

109
Q

Transfusion related acute lung injury (TRALI) is rare but potentially fatal reaction to blood product infusions that occurs within __ hours of transfusion. Even though TRALI can happen w/ any blood product, _____ have the greatest risk.

A

6 hours

plasma blood products, apheresis platelet concentrates and whole blood (rarely used today)

110
Q

Risk of iron overload has been recognized after packed red blood cell transfusions of more than __ units

A

20

111
Q

packed red blood cell transfusions are known to cause ___ electrolyte disturbance

A

hyperkalemia (from leakage of K+ out of the RBCs)

112
Q

Name the peripheral smear match with the disease:

  1. Target cell
  2. Howell-Jolly bodies:
  3. Heinz Bodies:
  4. Rouleaux formation:
  5. Poikilocytosis:
A
  1. Target cell: liver dz, hemoglobinopathy (thalassemia,)
  2. Howell-Jolly bodies: s/p splenectomy or asplenic pt
  3. Heinz Bodies: G6PD deficiency
  4. Rouleaux formation: elevated plasma proteins ie. myeloma
  5. Poikilocytosis: Extreme iron deficiency
113
Q

PTT is sensitive to what factors?

A

XII, XI, IX, VIII

114
Q

When mixing a pts prolonged PTT blood w/ 1:1 ratio w/ normal donors blood what does it mean if:

  1. PTT corrects:
  2. stays prolonged:
A
  1. corrects= def. of one of the factors (8, 9, 11, 12)

2. prolonged= a presence of an inhibitor (ie. lupus anticoagulant, factor VIII inhibitors, contamination w/ heparin)

PANCE (17 decks)

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