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Pathology Boards > Hematopathology > Flashcards

Hematopathology Flashcards

1
Q

Name the AML with recurrent genetic abnormalities that is associated with myeloblasts, monoblasts, promonocytes, and eosinophils with basophil-like granules? (favorable prognosis)

What coexisting mutation moves this leukemia from a favorable prognosis to an intermediate one?

A

AML with inv(16)(p13.1q22), CBFB-MYH11 fusion

KIT mutation

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2
Q

Name the AML with a favorable prognosis with monocytic differentiation and blasts that do not express CD34?

What coexisting mutation in this type moves it to a worse prognosis?

A

AML with mutated NPM1

FLT3-ITD

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3
Q

Name the AML with increased blasts and abnormal promyelocytes ?

What is the feared complication of this disease?

Describe the morphology of the two subtypes?

A

APL with t(15;17)(q22;q21) PML-RARA

Life-threatening coagulopathy with DIC

Hypergranular- Blasts with bilobed or convoluted nuclei, marked thrombocytopenia, abnormal promyelocytes with large granules

Hypogranular- Blasts with bilobed nuclei (sliding plates) and absent cytoplasmic granules

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4
Q

Name the AML with abnormal neutrophils with salmon-colored granules and dysplastic nuclear features?

What kind of prognosis does this have?

What confers a worse prognosis?

What is unique about the Auer rods in this disease?

What other disease is this associated with?

A

AML with t(8;21)(q22;q22) RUNX1-RUNX1T1

Favorable

Intermediate risk

They are long and thin with tapered ends

Systemic mastocystosis

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5
Q

Describe the molecular change associated with Follicular Lymphoma?

A

t(14;18)(q32;q21) BCL2-IGH

BCL2 is 18q21 and IGH is on chromosome 14

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6
Q

Name the AML with recurrent genetic abnormalities with a intermediate prognosis with mainly monoblasts and promonos in the peripheral blood containing azurophilic granules?

A

AML with t(9;11); MLLT3-KMT2A

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7
Q

Name the three AMLs with defining cytogenetics abnormalities (That is, if you have the abnormality, it has to be AML)

A

t(8;21), inv(16), t(15;17)

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8
Q

Name the four main differential diagnosis with microcytic anemia?

A

Iron deficiency anemia

Anemia of chronic disease

Thalessemia

Sideroblastic anemia

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9
Q

What does MCV stand for?

A

Mean cell volume

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10
Q

What does MCH stand for? Describe it!

What about MCHC? Describe it!

A

Mean corpuscular hemoglobin

The average amount of hemoglobin in a RBC

Mean corpuscular hemoglobin concentration

Hemoglobin concentration in a given volume of red cells

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11
Q

What is the physiological mechanism behind iron being stuck in the macrophage in anemia of chronic disease/inflammation?

A

Hepcidin blocks ferroportin in the macrophage, iron is stuck

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12
Q

Where is iron trapped in sideroblastic anemia?

A

The mitochondria

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13
Q

Describe ferritin in the context of an iron panel

A

Ferritin is a special storage protein that can squirrel iron away from pathogens. The level is increased in reactive conditions such as anemia of chronic disease, and decreased in iron deficiency anemia (you don’t want to store the little iron you have left)

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14
Q

Describe TIBC and transferrin in the context of an iron panel

A

TIBC measures the body’s ability to bind iron with transferrin. In iron deficiency anemia, this value is high as the liver produces more transferrin to maximize the little iron available.

In anemia of chronic disease, the TIBC is low as the body produces less transferrin and more ferritin (ferritin helps keep the iron away from bugs)

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15
Q

describe iron level in the context of an iron panel. What does a decreased level mean? Increased?

A

The iron level is a measure of the amount of iron bound to transferrin and is non-specific due to a considerable variation from person to person due to diet. Decreased levels are associated with both iron deficiency and anemia of chronic disease (does not measure iron bound to ferritin).
Increased levels can be associated with iron overload

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16
Q

Describe % transferrin saturation in the context of an iron panel?

A

This measures the percentage of transferrin that has iron bound to it. In iron deficiency anemia this level should be low as you have lots of transferrin but little iron. In anemia of chronic disease this value is typically normal. This is because the body is producing less transferrin and there is some iron around, it’s just that a lot of it is bound to ferritin.

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17
Q

What is sideroblastic anemia?

What is it’s most common cause?

What genetic syndrome is it associated with and how is this inherited?

A

Abnormalities in porphyrin blocks heme synthesis, iron gets stuck in the mitochrondria.

Alcohol

Erythroid specific ALA synthase (x-linked)

*Bonus, can also be associated with MDS

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18
Q

What does the iron panel look like in thalassemia minor?

What Hbg electrophoresis value will be changed? Increased or decreased?

A

Normal

HbA2, increased

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19
Q

Define megaloblastic anemia…

What is an interesting feature of the bone marrow in this condition?

A

Dysplasia

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20
Q

What is the common cause of B12 deficiency? Main clinical finding? Time of onset?

What is the common cause of folate deficiency?
Neurological symptoms? Time of onset

A

Anything causing malabsorption (pernicious anemia, etc..)

Neurological symptoms

Long time of onset

Decreased intake (leafy gree vegetables)

No, no neurological problems

Shorter time of onset

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21
Q

What happens to the levels of homeocytstein and MMA in folate deficiency?

In B12 deficiency?

A

Homecystein is increased and MMA is normal in folate deficiency

Both homocystein and MMA are increased in B12 deficiency

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22
Q

What is pernicious anemia?

What is the most specific antibody?

The most sensitive?

A

Autoimmune disorder with anti-intrinsic factor and anti-parietal cell antibodies. These cause chronic atrophic gastritis.

Anti-intrinsic is most specific (Intrinsically specific!)

Anti-parietal is most sensitive

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23
Q

The Schilling test is used for ____ not for ____

What is the concept?

A

Determine the cause of B12 deficiency, not to diagnose it

You give B12 with and without intrinsic factor and see if B12 is in the blood stream. If B12 alone is low and it’s normal with IF administration then you have pernicious anemia, if it is low and still low with IF then you either have malabsorption or bacterial overgrowth

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24
Q

Hemolytic anemia is defined as….

A

The abnormal breakdown of RBCs

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25
Q

What stain can be used to highlight reticulocytes?

A

Supravital blue stains like methylene blue

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26
Q

How are spherocytes formed?

What is the most common mutation in the hereditary form of spherocytosis?

A

RBCs lose some of their surface membrane, causing them to be unable to make the biconcave shape, and instead make a simple sphere.

Ankrin

27
Q

How are eliptocytes formed?

In order to diagnose hereditary eliptocytosis you need to have what percentage of eliptocytes?

A

They have cytoskeleton defects which prevent them from reforming their normal shape after squeezing through the capillaries.

> 25% eliptocytes

28
Q

Hereditary stomatocytosis is inherited in what manner? What defect is it associated with?

What other three conditions is it associated with?

A

AD

Defect in the Na/K permeability of RBC membrane

Alcohol, liver disease, Rh null disease

29
Q

What is a Heinz body? Bite cell?

What conditions are they associated with?

A

RBC inclusions made of hemoglobin

Bite cells are RBCs missing part of their membrane corresponding to where a Heinz body was removed by the spleen

Associated with G6PD deficiency and hemoglobin problems (unstable hb, alpha thal with Hb Barts or H)

30
Q

What does G6PD help with?

A

Prevents oxidative damage of the red cells via the pentose phosphate pathway

31
Q

How is G6PD inherited?

A

X-linked

32
Q

Echinocytes can be seen in what genetic abnormality? How is in inherited?

A

Pyruvate kinase deficiency

Autosomal recessive

33
Q

What are the four main conditions associated with acanthocytes?

A

Liver disease

Post splenectomy

McCloud Syndrome
(weak kell antigens)

Abetalipoproteinemia (mutated microsomal triglyceride transfer protein, can’t absorb fat from food)

34
Q

What state is iron in in Methemoglobins?

A

3+ (ferric) state

35
Q

Name the lanes from positive to negative in an alkaline gel?

Acid gel?

A

A F S C
A fat santa claus

F A S C
For a safe christmas

36
Q

HbA is composed of what types of chain(s)?

HbA2?

HbF?

A

Alpha2Beta2

Alpha2Delta2

Alpha2Gamma2

37
Q

What is considered a “fast moving” hemoglobin on alkaline gel? Name 4

What is considered a “slow moving” hemoglobin on alkaline gel? Name 1

A

Fast moving are at the positive end

Hb N, I, H and Barts (from the NIH!)

Slow moving are at the negative end

Constant Spring

38
Q

What runs in the C lane on Alkaline Gel? (4)

A

A CEO

HbA1, HbC (duh), HbE, HbO

39
Q

What runs in the S lane on alkaline gel?

5

A

HbS, HbD, HbG, HbLepore, hasharon

LSD G!!!

40
Q

Sickle cell disease is caused by…. (genetically)

Associated with what carcinoma?

A

A point mutation in the Beta-globulin chain (glutamic acid replaced by valine)

Renal medullary carcinoma

41
Q

What percentages of Hb should you have in sickle cell disease? Sickle cell trait?

A

HbS >80%, HbF <20%, HbA2 <4%, HbA =0

HbS 35-45%, HbF <2%m Hb A2 <4%, HbA 50-60%

42
Q

What does the morphology look like in the peripheral smear with sickle cell trait?

A

Normal

43
Q

What does the morphology look like in the peripheral smear with Hemoglobin C trait? Disease?

A

Target cells and crystals

Target cells and crystals (the same)

44
Q

What is the level of HbC in HbC trait? Disease?

A

50%

90%

45
Q

What is the physiological process behind the formation of target cells?

A

Too much cell membrane (opposite of spherocytes)

46
Q

Where do patients with HbE disease typically come from?

What lane does HbE run with on alkaline gel?

A

SE Asia

C (A CEO)

47
Q

What is HbH composed of? HbBarts?

What thalassemia are they associated with?

A

Beta(4) tetramers, gamma (4) tetramers

Alpha thalassemia

48
Q

Hemoglobin H forms what types of inclusion?

A

Golf-ball like inclusions

49
Q

What are the clinical symptoms of beta thalassemia minor? Intermedia? Major?

A

Minor- Asymptomatic with mild anemia

Intermedia- Anemia but not transfusion dependent

Major- Severe anemia, transfusion dependent

50
Q

What is paroxysmal nocturnal hemoglobinuria?

A

An acquired anemia due to a mutation that disrupts a protein in red cell membranes. This protein usually halts complement activation on red cells, and without it they are lysed intravascular.

51
Q

What is the gene mutation involved in paroxysmal nocturnal hemoglobinuria? What does it encode?

How can flow cytometry be used to detect this disease?

What is a classic test for this condition?

A

PIG-A, the GPI Anchor protein

Can look for the loss of GPI linked proteins

The HAM acidified serum lysis test

52
Q

Name the laboratory test that can be used to detect paroxysmal nocturnal hemoglobinuria?

How does it work?

A

The HAM acidified serum lysis test

Placing red blood cells in mild acid, they will lyse if they lack the proper enzyme

53
Q

What is a Howell-Jolly body?

What condition is it associated with?

A

A single dense inclusion in RBCs made of DNA

Seen post-splenectomy or auto-splenectomy

54
Q

What is basophillic stippling?

What conditions is it seen in?

A

Multiple blue dots composed of RNA

Lead poisoning, pyrimidine 5` nucleotidase deficiency

55
Q

What is a pappenheimer body?

What conditions is it associated with?

A

An inclusion in an RBC made of iron. Can be seen in iron overload and post splenectomy

56
Q

What tick infection is associated with babesia?

Where are the parasites located?

A

Ixodes spacularis

Inside and outside the red cells

57
Q

What is the mutation in Chediak Higashi syndrome? What does this cause?

What cells are effected by this?

A

CHS1 (LYST): Causes a defect in the lysosome formation causing decreased phagocytosis and microtubule polymerization

Cells with granules, PMNs, LGLs, neurons, platelets

Causes issues with those cell lines

58
Q

What are MYH9 related disorders?

How are they inherited?

What are they characterized by?

A

AD inherited disorders with large platelets but decreased overall platelets (thrombocytopenia), hearing loss, cataracts, WBCs with Dohle bodies, and glomerulonephritis

59
Q

What is Alder Reilly anomaly?

What morphologic feature is present in the WBCs in this disorder? How are the PMNs effected mechanically?

How is it inherited?

A

Lack the lysozymal enzymes to break down mucopolysaccarides

Dense azurophillic granules in ALL WBCs

No effect on function

AR

60
Q

Pelger-Huet anomaly is associated with what mutation?

What is the inherited form associated with clinically?

A

Lamin B receptor mutation

No clinical significance

61
Q

Contrast the macrophage morphology in Gaucher disease vs Niemann-Pick disease?

What deficiencies is each associated with respectively

A

Gaucher- Tissue paper cytoplasm with wrinkles, glucocerebrosidase deficiency

NP- Foamy vaculated cytoplasm, sphinomyelin deficiency

62
Q

What are the diagnostic criteria for LGL leukemia?

A

> 6 months with >2000 cells

63
Q

What are the 4 markers commonly expressed in hematogones?

What marker can help distinguish this from B ALL?

A

CD10, CD20, CD34, Tdt

B ALL should NOT have CD10

64
Q

What markers are associated with immature hematopoietic cells?

Myeloid cells?

Monocytic cells?

Megakaryocytes?

A

Immature- CD34, CD117, TdT

Myeloid: CD13, CD15, CD33

Monocytes: CD4, CD11b, CD14, CD24, CD64, CD68

Megakaryocytes: CD41, CD42, CD61

Pathology Boards (7 decks)

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