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HemeOnc Block 2 > Path HO2 > Flashcards

Path HO2 Flashcards

1
Q

Anemias of diminished erythropoiesis are all characterized by…

A

low reticulocyte count

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2
Q

Microcytic anemias are due to…

A

decreased Hgb production

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3
Q

Is the problem of microcytic anemias in the cytoplasm or in the nuclear maturation?

A

cytoplasmic maturation defect

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4
Q

What are four causes of microcytic anemia?

A
  1. iron deficiency anemia
  2. anemia of chronic disease
  3. sideroblastic anemia
  4. thalassemia
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5
Q

What is the MCC of anemia WW?

A

iron deficiency anemia

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6
Q

What are some causes of Fe deficiency anemia?

A

inadequate intake

impaired absorption

increased requirement

chronic blood loss

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7
Q

Fe deficiency in post-menopausal women and adult men is due to…

A

chronic blood loss/cancer until proven otherwise

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8
Q

Where does absorption of Fe occur?

A

duodenum

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9
Q

What cells in the duodenum help transport Fe across their cell membrane?

A

enterocytes

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10
Q

What transporter do enterocytes use to transport Fe across their cell membrane and into the blood?

A

ferroportin

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11
Q

After Fe is inside an enterocyte, what transports the Fe through the blood so that it can reach liver and bone marrow?

A

transferrin

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12
Q

What is stored intracellular Fe bound to? Why does it need to be bound?

A

ferritin

prevents iron from forming free radiacals via the Phenton reaction

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13
Q

Fe absorption is regulated by plasma levels of …

How does this work?

A

hepcidin

inhibits Fe transfer from enterocytes to plasma by binding to ferroportin causing it to be endocytosed and degraded

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14
Q

What are specific PE findings of Fe deficiency anemia?

A

koilonychia

alopecia

atrophic glossitis

atrophic gastritis

intestinal malabsorption

pica

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15
Q

80% of functional Fe is found where?

A

in hgb

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16
Q

What are some morphologic findings on a peripheral blood smear of Fe deficiency anemia?

A

microcytic hypochromic

anisocytosis (increased RDW)

“pencil cells”

low reticulocyte count

mild thrombocytosis

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17
Q

In Fe deficiency anemia, what will the Fe labs look like?

A

low ferritin (marrow Fe)

increased TIBC (total iron binding capacity)

low serum iron

low % saturation

increased Serum soluble Transferrin Receptor

increased Free erythrocyte protoporphyrin (FEP)

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18
Q

Anemia of chronic disease is due to…

A

defective iron cycling between macrophages and developing RBCs, resulting decrease in erythropoiesis

no intrinsic defect of RBCs

mediate by cytokines

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19
Q

What is the MC type of anemia in hospitalized pts?

A

anemia of chronic disease

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20
Q

How does hepcidin sequester iron in storage sites?

A

limiting iron transfer from macrophages to erythroid precursors

suppressing erythropoietin production - prevent bacteria from accessisng iron needed for their survival

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21
Q

What are morphological characteristics of anemia of chronic disease?

A

NONE

reticulocyte count is low

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22
Q

What are the lab findings with anemia of chronic disease?

A

decreased serum Fe

decreased TIBC

decreased % saturation

normal serum soluble transferrin receptors

increased ferritin (marrow Fe)

increased Free erythrocyte protoporphyrin (FEP)

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23
Q

anemia caused by abnormal fe metabolism within the RBC itself is called what? Going a little bit more in depth, what is happening?

A

sideroblastic anemia

defective protoporphyrin synthesis

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24
Q

What causes the look of ‘ringed sideroblasts’ ?

A

iron is sequestered in the developing RBC mitochondria and can’t be used for heme synthesis

iron swells and distorts the mito (which is surrounding the nucleus) showing a ring of dark blue staining, Fe containing mito around the nucleus

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25
Q

What are the MCC of sideroblastic anemia?

A

acquired causes like myelodysplastic syndromes or secondary to toxic insults like lead, drugs, or alcohol

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26
Q

What will the lab findings be in sideroblastic anemia?

A

increased serum Fe

decreased or nl TIBC

increased % saturation

variable SS Transferrin receptor

increased ferritin (marrow Fe)

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27
Q

megaloblastic marrow can be caused by what three things?

A

folate deficiency

Vt B12 deficiency

drugs that interfere with DNA synthesis

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28
Q

How does Vit B12 or folate deficinecy cause macrocytic megaloblastic anemia?

A

they are coenzymes required for the synthesis of thymidine - without, DNA synthesis is impaired

results in defective nuclear maturation of rapidly proliferating cells

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29
Q

long standing, untreated B12 deficiency eventually results in …

A

subacute combined degeneration of the spinal cord by demyelination

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30
Q

what are some causes of vit B12 deficiency?

A

strict vegetarianism

intrinsic factor deficiency

broad fish tape worm - diphyllobothrium latum

pregnancy

hyperthyroidism

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31
Q

Does it take longer for a deficiency in vit B12 or folate take longer to cause symptoms?

A

folate deficiency will be noticed within a few months

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32
Q

what is a particular form of megaloblastic anemia due to autoimmune gastritis, destruction of parietal cells and B12 deficiency d/t lack of intrinsic factor?

A

pernicious anemia (PA)

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33
Q

in peripheral blood smear, what morphologies will you see with megaloblastic macrocytic anemia?

A

anisopoikilocytosis

macro-ovalocytes

neutrophils larger with hypersegmented nuclei

rare tear drop cells

reticulocyte count is low

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34
Q

What are unique labs you might find elevated with Vit B12 deficiency anemia?

A

serum homocysteine and methylmalonic acid

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35
Q

Methylmalonic acid will be… in folate deficiency anemia

A

normal

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36
Q

chronic primary hematopoietic failure with pancytopenia is considered…

A

aplastic anemia

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37
Q

what will the morphology of aplastic anemia appear like?

A

blood: typically pancytopenia, no specific morph abnls

bone marrow: markedly hypocellular, largely devoid of hematopoietic cells; mainly contains fat and stromal cells

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38
Q

what is a primary marrow disorder in which only erythroid progenitors are suppressed?

A

pure red cell aplasia

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39
Q

What are some common etiologies of pure red cell aplasia?

A

neoplasms, esp thymoma and large granular lymphocytic leukemia

autoimmune diseases

parvovirus B19 (in bone marrow, rare giant erythroblasts are seen, with intranuclear inlcusions)

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40
Q

Space occupying lesions in the marrow replace hematopoietic tissue is called what?

what is this caused by?

A

myelophthisic anemia

caused by metastatic carcinoma

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41
Q

the only congenital disorder characterized initially by isolated and sustained (pure) red cell aplasia

A

diamond-blackfan anemia

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42
Q

What are clinical features of Diamond-Blackfan anemia? What does the CBC look like?

A

short stature, craniofacial and thumb skeletal abnls

CBC: macrocytic anemia with elevated HbF and increased RBC adenosine deaminase; markedly decreased reticulocyte count

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43
Q

hemolytic anemias are usually (intravascular/extravascular) meaning they take place…

A

extravascular

in the macrophages of the reticuloendothelial system

44
Q

If bone marrow is appropriately responding to hemolytic anemia, what will happen?

A

erythroid hyperplasia and peripheral blood reticulocytosis

45
Q

physiologic destruction of RBCs is …

this takes place where?

A

extravascular hemolysis

takes place within the macrophages of liver, spleen, and marrow

46
Q

What breakdown products does extravascular hemolysis create?

A

heme ( to biliverdin and then bilirubin)

globin (degraded amino acid compounds)

iron (taken up by RE cells and recycled)

lactate dehydrogenase (LDH)

47
Q

What are common clinical features of extravascular hemolytic anemia?

A

anemia

splenomegaly

jaundice

variable decreases in serum haptoglobin

48
Q

Intravascular hemolysis is never physiological but is always…

A

pathological

49
Q

What does intravascualr hemolysis release from RBCs?

A

free hemoglobin (into the plasma which then binds haptoglobin, normally is eaten by macrophages, but if overwhelmed, will cause hemosiderinuria and if even more overwhelmed will cause hemoglobinuria)

free hemoglobin could also bind to transferring and albumin resulting in methhemoglobin

50
Q

Splenomegaly is NOT a feature of…

A

intravascular hemolysis

51
Q

What are common clinical features of intravascular hemolysis?

A

anemia

hemoglobinemia

hemoglobinuria

hemosiderinuria

decreased plasma haptoglobin**

jaundice

52
Q

In all hemolytic anemias, reticulocytes will be increasingly released into peripheral blood appearing as…

A

polychromasia on Wright-Giemsa stains (increased MCV and blue-tinted cytoplasm)

reticulocytosis on new methylene blue stain

53
Q

In other organs, hemolytic anemias will cause:

A

hemosiderosis (esp within spleen, liver, and bone marrow

extrameduallary hematopoiesis

pigmanet gallstones and cholelithiasis

54
Q

alpha thalassemia is mainly from…

A

gene deletions

55
Q

beta thalassemia is mainly from …

A

point mutations that lead to defects in transcription, splicing or translation of beta globin mRNA

56
Q

if all four alpha genes are deleted in alpha thalassemia, what results? What is this called hgb wise?

A

hydrops fetalis, fatal in utero

hgb barts, four gamma chains instead of alpha

57
Q

how are beta thalassemias divided?

A

major

intermedia

minor

58
Q

What is the predominant form of hgb in B thalassemia major? When is this disease apparent?

A

HbF

first 6-9 months of life

59
Q

In B thalassemia major, what will be seen on XRs?

A

crew cut appearance on skull

60
Q

If there are three deletions of alpha globins in thalassemia, what occurs?

A

HbH disease, resembling beta thalassemia intermedia

61
Q

What will Fe lab studies in thalassemia show?

A

increased serum Fe

decreased or nl TIBC

increased or normal % saturation

variable SS Transferrin receptor

increased or normal ferritin (marrow Fe)

62
Q

reticulocyte count is …. in thalassemia

A

increased

63
Q

Sickle cell anemia

A

single point mutation in gene for beta chain of hgb

glu 6 to val

may progress to functional asplenia, then more susceptible to infection by encapsulated organisms

osteomyelitis caused by salmonella species

64
Q

heterozygotes for HbS is thought to confer protection against…

A

P. falciparum

65
Q

hereditary spherocytosis

A

cytoskeleton defect

sphere shape causes them to get trapped in cords (erythrostasis) and then phagocytosed by macrophages

normochromic normocytic anemia and increased MCHC on CBC

66
Q

How do you diagnose hereditary spherocytosis?

A

osmotic fragility test.

spherocytes rupture easily in a hypotonic solution

67
Q

G6PD deficiency

A

susceptible to oxidative stress (hemolysis only after such stress)

X linked

may confer protection v Plasmodium falciparum

hemolysis is both extravascular and intravascular (Heinz bodies in cells, then taken out, now have bite cells)

can not do enzyme testing during an episode may yield false negative result

68
Q

What are three types of antibodies that can cause immune hemolytic anemia?

A

autoantibodies (cold and warm)

alloantibodies

drug-related antibodies and MOA

69
Q

Cold and warm agglutinins go with what immunoglobulins?

A

IgG - warm

IgM - cold

70
Q

What is the only acquired stem cell defect to cause hemolytic anemia? (intrinsic to RBC)

A

Paroxysmal Nocturnal Hemoglobinuria

71
Q

What is the pathogenesis behind PNH?

A

partial or complete loss of linkage of cell surface proteins to the membrane by GPI anchors due to mutations of the PIGA gene

72
Q

How would I diagnose PNH?

A

loss of CD55 (DAF) and CD59 (MIRL) on flow cytometry

73
Q

What may be causes of decreased extravastation of cells from the blood to the tissues, causing leukocytosis?

A

glucocorticoids

74
Q

What is the number one cause of neutrophilia?

A

acute bacterial infections/sepsis

75
Q

What is a leukemoid reaction?

A

severe, persistant, reactive neutrophilic leukocytosis above 50k/uL when the cause is not leukemia

often caused by severe infection, toxic reactions, malignancies, severe hemorrhage, or acute hemolysis

76
Q

leukoerythroblastosis is… and is typically caused by …

A

immature myeloid and erythroid precursors in peripheral blood

myelophthisis (bone marrow disruption/inflitration by tumor and/or fibrosis)

77
Q

Eosiniophilia is caused by

A

allergic disorders

drug hypersenstivities

parasitic infections

some neoplasms

78
Q

Basophilia can be from neoplastic or non-neoplastic conditions, but it is most likely …

A

neoplastic

79
Q

Monocytosis is often from…

A

chronic infections

bacterial endocarditis

some malignancies

recovery from bone marrow suppression (chemo)

80
Q

In non-neoplastic causes of neutropenia, what happens in each?

A
  • proliferation defect
    • agranulocytosis
    • myeloid hypoplasia
  • maturation defect
    • myeloid hyperplasia
  • survival defect
    • early destruction after maturation
    • myeloid hyperplasia
  • distribution abnormality
    • sequestration in spleen
    • bone marrow is variable
81
Q

potenital consequence of severe neutropenia is..

A

infections, esp candida and aspergillus spp.

82
Q

Lymphocytopenia could be caused by…

A

congenital deficiency

infectious disease

iatrogenic

malnutrition

83
Q

Monocytopenia causes include

A

aplastic anemia

hairy cell leukemia

corticosteroids

acute infections with endotoxemia

84
Q

Reactive plasmacytosis DOES NOT mean…

A

plasma cell neoplasm

85
Q

MCC of reactive lymphoid aggregates are

A

infections, drug therapy, immune disorders

86
Q

Pelger-Huet anomaly

A

nuclear hyposegmentation of neutrophils and other granulocytes

autosomal dominant

inherited form is not clinically relavent since neutrophils are still functional

acquired is often non-functional and hypogranular

87
Q

May-Hegglin anomaly

A

asymptomatic (mostly). autosomal dominant

MYH9RD

large platelets

thrombocytopenia in spite of adequate numbers of bone marrow megakaryocytes, normal morphology

sensorineural hearing loss

large dohle body-like inclusions in neutrophils

88
Q

Chediak- Higashi syndrome

A

severe anomaly with giant granules in neutrophils, eosinophils, monocytes, and lymphocytes

autosomal recessive

children have neutropenia, thrombocytopenia, and recurrent infections, esp to staph aureus

most die in childhood from infections or bleeding

89
Q

Alder-Reilly anomaly

A

morphologic result of a type of lysosomal storage disease (LSD)

deficiency of any one of several lysosomal enzymes, resulting in accumulation of mucopolysaccharides, glycosphinglipids or glycoproteins within lysosomes

autosomal recessive

coarse facies, cardiac abnl, skeletal abnl, developemental delay, hepatosplenomegaly, corneal clouding

cytopenias may be present

prominent azurophilic granules are seen in leukocytes NOT related to infection

90
Q

Bernard- Soulier syndrome

A

mutations in GPIb cause decreased membrane GP Ib-IX, a receptor for vWF

bleeding due to defective adhesion of platelets to subendothelial collagen after vascular injury

similar to May-Hegglin with large platelets, but here they actually have impaired function

91
Q

Thrombocytopenia with absent radii (TWAR)

A

autosomal recessive

normal platelet morphology but abnl function

decreased megakaryocytes in bone marrow

absent radii, abnormal thumbs?

GI or skeletal anomalies at birth

92
Q

Wiscott-Aldrich Syndrome

A

X linked

thrombocytopenia with small platelets

immunodeficiency due to failure of lymphocyte maturation

often involves IgM deficiency

93
Q

Glanzman thrombasthenia

A

thrombasthenia “lazy” platelets

normal platelet count and morph but abnl function

dysfunction of glycoprotein IIb-IIIa causing defective platelet aggregation

thrombathenic platelets react only to ristocetin and failr to aggregate in response to ADP from collagen

rarely acquired as autoimmune disorder

94
Q

primary immune thrombocytopenia

A

acquired, otherwise asymptomatic adults

insidious with mucocutaneous bleeding

absense of systemic symptoms and absence of splenomegaly help make the dx

bone marrow (if done) is normal, with plenty of megakaryocytes with normal morph

largely a dx of exclusion

95
Q

Secondary ITP is associated with…

A

autoimmune diseases, lymphomas, viral infections, and drugs

96
Q

formation of widespread microvascular thrombi after activation of coagulation cascade by tissue factor a mimic, resulting in microangiopathic hemolytic anemia and thrombocytopenia

A

DIC

97
Q

What are coagulation factors and other things like in labs of DIC pts?

A

fibrinogen is low

D-dimer and FDP are high

PT, PTT, and TT may be prolonged or somestimes normal in early stages

schistiocytes are seen on peripheral blood smear

98
Q

Thrombotic thrombocytopenia purpura/ hemolytic uremic syndrome

A

related disorders characterized by diffuse microvascular occlusion of arterioles and capillaries by thrombi mainly composed of platelets, not fibrin

no perivascular inflammation

clinical ssx overlap with DIC, preeclampsia, HELLP, malignant HTN, and vascultiis

COAGULATION SCREENING TESTS ARE USUALLY NORMAL

99
Q

What kind of treatment does TTP require?

A

NOT PLATELETS

emergent plasma apheresis with FFP or cryo-poor plasma

100
Q

What is the MCC of secondary HUS?

A

infection - shiga like toxin

101
Q

How do you treat HUS?

A

supportive with dialysis

102
Q

What type of heparin induced thrombocytopenia occurs immediately after heparin admin?

A

type 1

103
Q

How does type 2 HIT work?

A

occurs within 5- 14 days after beginning heparin

caused by autoantibodies to a complex of heparin and LMW proteins, such as platelet factor 4

104
Q

What are two clinical clues to HIT?

A

fall in platelet count around 1-2 weeks after starting heparin

unexpected shortening of the APTT in those receiving therapeutic concentrations of heparin

105
Q

What are the affects of asa on platelet function?

A

impairs txa2 dependent platelet aggregation bc of inhibiting COX (1>2)

asa has no affect on the coagulation cascade

platelets return to normal within 5-6 days after last ASA dose

106
Q

Pseudothrombocytopenia

A

platelet aggregates from traumatic blood draw or inadequate mixing the MCC

could be due to EDTA induced aggregation, draw in heparinized tube to get accurate platelet count

HemeOnc Block 2 (3 decks)

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