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Step 2 CK > Hematology > Flashcards

Hematology Flashcards

1
Q

What symptoms should you expect with the following Hematocrit:

25-30%

20-25%

<20%

A

25-30% = Dyspnea and fatigue

20-25% = Lightheadedness, angina

<20% = Syncope and chest pain

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2
Q

Causes of low MCV

A

Iron deficiency

Thalassemia

Sideroblastic anemia

Anemia of chronic disease

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3
Q

What is the only microcytic anemia to have an elevated reticulocyte count?

A

alpha thalassemia with 3 genes deleted

all other microcytic anemias have a low reticulocyte count

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4
Q

Cause of high MCV

A

B12 and folate deficiency

Sideroblastic anemia

Alcoholism

Liver disease or hypothyroidism

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5
Q

Macrocytic anemias all give what type of reticulocyte count?

A

Low

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6
Q

At what hematocrit do you transfuse a patient?

A

If the patient is symptomatic (SOB, lightheaded/confused, hypotensive/tachycardic, chest pain) = transfuse

If the hematocrit is very low (25-30) in an elderly patient or one with heart disease = transfuse

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7
Q

Each unit of PRBCs should raise the hematocrit by?

A

3 points per unit

Will raise the Hgb by 1 per unit

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8
Q

When is FFP NOT the answer?

A

NOT a choice for hemophilia A or B or von Willebrand disease

Used to replace clotting factors (elevated PT, aPTT, or INR)

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9
Q

Cryoprecipitate is used to replace

A

fibrinogen

Has some utility in DIC (high levels of factor VII and VWF)

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10
Q

When do you give platelets?

A

To a bleeding patient when platelet count is <50,000

CONTRAINDICATED for TTP and HUS

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11
Q

Where is iron absorbed in the body?

A

Duodenum (4 mg/day)

  • Body needs roughly 1-2 mg/day*
  • Menstruating women need 2-3 mg/day*
  • Pregnant women need 5-6 mg/day*
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12
Q

What is hepcidin?

A

Regulates iron absorption

Hepcidin levels are low in anemia of chronic disease

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13
Q

What is the most likely diagnosis for anemia with:

  1. Blood loss
  2. Menstruation
  3. Cancer
  4. RA
  5. Alcoholic
  6. Asymptomatic
A
  1. Iron deficiency
  2. Iron deficiency
  3. Chronic disease
  4. Chronic disease
  5. Sideroblastic
  6. Thalassemia
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14
Q

Target cells are seen most commonly with

A

Thalassemia

However, they can be seen with all causes of microcytic anemia

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15
Q

Anemia with iron studies showing:

  1. Low ferritin
  2. High iron
  3. Normal iron
A
  1. Iron deficiency
  2. Sideroblastic anemia
  3. Thalassemia
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16
Q

TIBC in Iron deficiency vs Chronic disease

A

Low TIBC = chronic disease

High TIBC = iron deficiency

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17
Q

Most accurate test for:

Iron deficiency

Sideroblastic anemia

Thalassemia

A

Iron deficiency = bone marrow biopsy for stainable iron which is decreased (rarely done)

Sideroblastic anemia = prussian blue staining for ringed sideroblasts

Thalassemia = hemoglobin electrophoresis (exception = alpha thalassemia, which is diagnosed by DNA analysis)

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18
Q

Most appropriate next step when MCV is elevated

A

Peripheral blood smear

Only B12 and folate deficiency (and antimetabolite medications) cause hypersegmentation

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19
Q

Pancreatic insufficiency causes what type of anemia

A

Macrocytic (need pancreatic enzymes to remove B12 from the R-protein so it can bind with IF)

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20
Q

When the vignette suggests B12 deficiency, but the B12 level is equivocal, what is the next step?

A

MMA level (only elevated with B12 deficiency)

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21
Q

What is a complication of rapid B12 or folate replacement?

A

Hypokalemia

There is no other condition in which cells are generated so rapidly that they use all the potassium

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22
Q

Bilirubin gallstones

Osteomyelitis

Retinopathy

Stroke

Skin ulcers

Avascular necrosis

A

Common manifestation of SCD

Children present with dactylitis (inflammation of fingers)

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23
Q

The best initial test for SCD?

A

Peripheral smear

Sickel cell trait (AS disease) does NOT give sickled cells

The most accurate test is hemoglobin electrophoresis

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24
Q

What lowers mortality in SCD?

A

Hydroxyurea (prevents recurrences by increasing Hgb F)

Antibiotics (ceftriaxone, levofloxacin, or moxifloxacin) with fever

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25
Q

Exchange transfusion for SCD is used if there is severe vasoocclusive crisis presenting with:

A

Acute chest syndrome

Priapism

Stroke

Visual disturbance from retinal infarction

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26
Q

Best initial test for a patient with SCD with a decreasing hematocrit?

A

Reticulocyte count (first clue to parvovirus)

Patients with SCD have very high reticulocyte counts because of chronic compensated hemolysis. Parvovirus B19 causes an aplastic anemia crisis which freezes the growth of the marrow.

Most accurate test for parvo is a PCR for DNA. IVIG = best initial therapy

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27
Q

What is the only manifestation of sickle cell trait?

A

Defect in the ability to concentrate the urine (isosthenuria)

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28
Q

Most accurate test for Hereditary Spherocytosis

A

Osmotic fragility

Defect in cytoskeleton leading to abnormal round shape and loss of flexibility

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29
Q

Treatment for Hereditary Spherocytosis

A

Chronic folic acid replacement

Splenectomy to stop hemolysis

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30
Q

Treatment of Warm Hemolysis vs Cold Agglutinin Disease

A

Warm = glucocorticoids, then splenectomy, then IVIG, then rituximab

Cold = stay warm, rituximab (steroids and splenectomy DO NOT WORK)

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31
Q

Cyclophosphamide

Cyclosporine

Azathioprine

Mycophenolate mofetil

A

Alternative treatments to diminish the need for steroids

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32
Q

Heinz bodies and bite cells

A

Seen in G6PD deficiency

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33
Q

Both TTP and HUS are characterized by:

A

Intravascular hemolysis with fragmented red cells (schistocytes)

Thrombocytopenia

Renal insufficiency

TTP is also associated with neurological disorders and fever

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34
Q

What is paroxysmal nocturnal hemoglobinuria?

A

A clonal stem cell defect with increased sensitivity of red cells to complement in acidosis leading to aplastic anemia, myelodysplasia, or acute leukemia

Deficiency of CD 55 and 59 (decay accelerating factor)

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35
Q

Most common cause of death from PNH

A

Thrombosis

Mesenteric and hepatic veins are the most common sites

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36
Q

Define aplastic anemia

A

Pancytopenia of unclear etiology

Acts as an autoimmune disorder in which the T cells attack the patient’s own marrow

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37
Q

Treatment for aplastic anemia

A

allogenic bone marrow transplantation (BMT)

if the patient is over 50 or there is no matched donor, the treatment is antithymocyte globulin (ATG) and cyclosporine

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38
Q

Define polycythemia vera

A

Unregulated overproduction of all 3 cell lines, but red cell overproduction is the most prominent

Mutation in the JAK2 protein (red cells grow wildly despite a low EPO level)

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39
Q

Hypertension

Heptosplenomegaly

Bleeding and Thrombosis

Pruritis following warm showers

A

Polycythemia vera

Up to 40% of patients with PV have gout

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40
Q

Most accurate test for PV

A

JAK2 mutation

Also look for elevated hematocrit (>60), low EPO, and elevated B12

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41
Q

Treatment for PV

A

Phlebotomy and ASA to prevent thrombosis

Hydroxyurea helps lower the cell count

Allopurinol or rasburicase protects against uric acid rise

Antihistamines

Target Hct is < 45%

42
Q

Apart from PV, what is a JAK2 mutation also associated with?

A

Essential thrombocytosis

43
Q

What is myelofibrosis?

A

Disease of older persons with a pancytopenia associated with a bone marrow showing marked fibrosis; *blood production shifts to the spleen and liver (enlarged)

Teardrop-shaped cells and nucleated RBCs

*Extramedullary hematopoiesis

44
Q

Lab values indicative of hemolysis for normocytic anemia

A

Increased LDH

Decreased haptoglobin

Increased Tbili

Not seen in hemorrhage (exception = production problem + hemolysis)

45
Q

Why is iron increased in sideroblastic anemia?

A

Iron gets stuck in the mitochondria; cells cannot use the iron, so the body keeps sending more that also gets stuck

46
Q

Reversible and Irreversible causes of sideroblastic anemia

A

Reversible = drugs, EtOH, and lead

Irreversible = B6 deficiency (cannot just replace) and MDS

47
Q

What will the hemoglobin electrophoresis show for alpha thalassemia?

A

Nothing (i.e., it will be normal)

48
Q

What must you consider when treating thalassemia?

A

Chronic transfusion = iron overload

Managed with deferasirox or deferiprone (PO iron chelators)

Deferoxamine is a parenteral version

phlebotomy is NEVER the answer

49
Q

IgA attacks parietal cells

A

Pernicious anemia (leads to B12 deficiency d/t lack of IF)

50
Q

How long is B12 and folate stored in the body?

A

B12 = 3-10 years (from animal products)

Folate = 3-6 weeks (from leafy greens)

51
Q

Is the peripheral neuropathy from B12 deficiency reversible with replenishment?

A

No

52
Q

What must you consider when replacing B12?

A

Whether to give IM or PO

Absorption problem (pernicious anemia, crohn’s, or gastric bypass) = IM

B12 deficient patients = PO

Schilling’s test helps distinguish between the two

53
Q

Why is cow’s milk problematic in infants <1 or when consumed excessively before the age of 2?

A

Low in iron

Also causes occult intestinal blood loss

Result = Iron deficiency anemia

54
Q

The most frequently tested type of acute leukemia

A

M3 or acute promyelocytic leukemia

this is because it is associated with DIC

55
Q

Best initial test when suspecting leukemia

A

Blood smear (showing blasts)

Most accurate test = flow cytometry

56
Q

Myeloperoxidase positive

A

AML

57
Q

What translocation is associated with M3?

A

15 and 17

58
Q

What are Auer rods and what are they associated with?

A

Eosinophilic inclusions associated with AML

M3 is the most common associaton

59
Q

Tdt positive

A

ALL

Also cALLa positive

60
Q

Unique treatment for ALL and M3

A

ALL = add intrathecal methotrexate to prevent relapse in the CNS

M3 = add all-trans-retinoic acid (ATRA)

Both AML and ALL are treated with chemo to remove blasts and induce remission

61
Q

BCR-ABL

9:22 translocation

Philadelphia chromosome

A

All synonymous for CML

62
Q

Which myeloproliferative disorder has the greatest likelihood of transforming into acute leukemia (blast crisis)?

A

CML

If untreated, 20% of patients per year

63
Q

Treatment for CML

A

Translocation of 9:22 results in overlyactive tyrosine kinase

Tryosine kinase inhibitors = imatinib, dasatinib, or nilotinib

Only a BMT can cure CML, but this is never the initial therapy

64
Q

What is the most important first step in acute leukostasis reaction?

A

Leukapheresis (removing the excessive white cells)

65
Q

Define myelodysplastic syndrome

A

MDS is a preleukemic disorder presenting in older patients with a pancytopenia despite hypercellular bone marrow

66
Q

5q deletion

A

characteristic abnormality of MDS

5q deletion has an excellent response to lenalidomide

Also look for increased MCV, nucleated red cells, and ringed sideroblasts

67
Q

Pelger-Huet cell

A

most distinct lab abnormality in MDS (hyposegmented nucleus in WBC)

68
Q

Treatment for CLL

A

Most people die with it, not from it

Stage III (anemia) and Stage IV (thrombocytopenia) are treated with fludarabine, cyclophosphamide, and rituximab

69
Q

Massive splenomegaly

“Dry” tap

A

Hairy Cell Leukemia

A smear will show hairy cells; flow cytometry will show CD11c

Treat with cladribine or pentostatin

70
Q

Best next step when patient presents with:

Painless lymphadenopathy

“B” symptoms

A

B symptoms = fever, wt loss, drenching night sweats

Next step = excisional biopsy

This is characteristic of NHL and HD

Most common wrong answer is needle aspiration

71
Q

How to stage NHL?

A

CXR, if negative then CT chest/abd/pelvis, if negative then BM Bx

Stage 1 = 1 lymph node group

Stage 2 = 2 or more on the same side of the diaphragm

Stage 3 = both sides of the diaphragm

Stage 4 = widespread disease

72
Q

How to treat advanced NHL (stage III or IV)?

A

R-CHOP

Rituximab (antibody against CD20)

Cyclophosphamide

Hydroxydaunorubicin (aka adriamycin or doxorubicin)

Oncovin (aka vincristine)

Prednisone

Note: Unlike HD, NHL presents in advanced stages in 80-90% of cases

73
Q

Reed-Sternberg cells

A

aka lacunar histiocytes

Synonymous for Hodgkin Disease

74
Q

Pel-Epstein (cyclical) fevers

Alcohol = painful lymphadenopathy

A

HD

75
Q

Burkitt’s lymphoma

Extranodal disease

A

NHL

76
Q

Treatment of HD

A

Stage I and II (80-90%) = local radiation with chemo

Stage III and IV = ABVD

Adriamycin (doxorubicin)

Bleomycin

Vinblastine

Dacarbazine

77
Q

When is adriamycin (doxorubicin) contraindicated?

A

Adriamycin is cardiotoxic

Cannot use it when EF is < 50% (best determined with MUGA or nuclear ventriculogram which is more accurate than an echo)

78
Q

Adverse Effects of:

Doxorubicin

Vincristine

Cyclophosphamide

Cisplatin

A

Chemo-man

Doxo = cardiomyopathy

Vin = peripheral neuropathy

Cyclo = hemorrhagic cystitis

Cisplatin = renal and ototoxicity

79
Q

Most common presentation for multiple myeloma?

A

Bone pain from pathologic fracture (bone breaks under what would be considered normal use)

80
Q

Most common cause of death in myeloma?

A

Renal failure and infection

RF d/t accumulation of immunoglobulins and Bence-Jones protein in the kidney (also 2/2 hypercalcemia and hyperuricemia)

81
Q

Anion gap in myeloma?

A

Decreased anion gap

IgG is cationic (will increase chloride and bicarbonate levels)

82
Q

SPEP for myeloma

A

IgG (60%)

IgA (25%)

Although these are all “M” spikes, remember IgM is a separate disease called Waldenstrom macroglobulinemia

83
Q

What is the single most accurate test for myeloma?

A

Bone marrow biopsy showing > 10% plasma cells

The most common wrong answer is SPEP (99% of people with an “M-spike” do NOT have myeloma)

84
Q

MGUS

A

IgG or IgA spikes on an SPEP without myeloma

1% a year transform into myeloma

85
Q

Best initial therapy for Waldenstrom Macroglobulinemia

A

Plasmapheresis

IgM spike results in hyperviscosity

86
Q

What is ITP

A

Diagnosis of exclusion

Look for isolated thrombocytopenia, normal-sized spleen, increased megakaryocytes, or antiplatelet antibodies

Treat with prednisone

87
Q

Before splenectomy, what vaccinations must you give?

A

N meningitidis

H influenza

Pneumococcus

88
Q

Best therapy for VWD?

A

DDAVP (desmopressin), which releases subendothelial stores of VWF

Look for bleeding related to platelets that is markedly worse after taking aspirin

89
Q

Increased PT and aPTT

Low platelet count

Elevated d-dimer and fibrin split products

Decreased fibrinogen

A

Characteristic findings for DIC

90
Q

How to confirm HIT

A

ELISA for platelet factor 4 (PF4) antibodies or the serotonin release assay

91
Q

Treatment for HIT

A

Stop all heparin-containing products

Administer direct thrombin inhibitors: argatroban, lepirudin, bivalirudin, and fondaparinux

92
Q

2 main antiphospholipid syndromes

A

Both cause thrombosis

  1. Lupus anticoagulant (associated with elevated aPTT)
  2. Anticardiolipin (associated with multiple spontaneous abortions)
93
Q

Only cause of thrombophilia with an abnormality in the aPTT

A

APL syndrome

Requires lifelong anticoagulation

Best initial test = mixing study (aPTT will remain elevated even after the mix)

The anticoagulation effect only exists in vitro; in vivo (i.e., patients) it results in increased clot formation NOT bleeding disorders

94
Q

Fever

Anemia

Thrombocytopenia

Renal Fx

Neuro Sxs

A

Think TTP

Symptoms = “FAT-RN”

95
Q

Causes of thrombocytopenia

A

“Alphabet Soup”

HIT

TTP

DIC

ITP

96
Q

Pt > 70

Hypercalcemia

Renal Fx

Anemia

Bone Pain

A

MM

Get skeletal survey to look for lytic lesion (caused by osteoclast activating factor - OAF), not a bone scan

97
Q

SPEP (+)

UPEP (-)

Skeletal (-)

BM Bx < 10%

A

MGUS

98
Q

SPEP (+)

UPEP (-)

Skeletal (-)

BM Bx > 10% lymphoma (not plasma)

A

Waldenstrom Macroglobulinemia

99
Q

Treatment for ITP

A

Steroids

then IVIG

then Rituximab

100
Q

Bernard Soulier

A

GlyIb deficiency

101
Q

Glanzman thromobocytopenia

A

GlyIIbIIIa deficiency

Step 2 CK (18 decks)

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