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Pathophysiology > Hematopathology > Flashcards

Hematopathology Flashcards

0
Q

EPO

A

Erythropoietin

  • Activates erythroid progenitor cells
  • Released by KIDNEY interstitial peritubular cells in response to hypoxia
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1
Q

What is the composition of bone marrow?

A

cellularity is the ratio of hematopoeitic cells to fat

varies w/ age

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2
Q

Trilineage hematopoiesis

A

Bone marrow consists of:

granulocytic precursors
erythroid precursors
megakaryocytes

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3
Q

TPO

A

Thrombopoietin

  • facilitates production and maturation of MEGAKARYOCYTES
  • produced by LIVER AND KIDNEY
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4
Q

What does a left shift mean?

A

Leukoerythroblastic reaction (immature cells getting out too soon)

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5
Q

Left shift

A

shift toward immature leukocytes

  • significant number of bands and neutrophils
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6
Q

How do we evaluate bone marrow?

A

iliac crest - needle core biopsy/aspiration

anterior tibia - source for infants

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7
Q

Cytoskeleton of red blood cell - Horizontal

A

Spectrin heterodimers

Spectrin-actin-protein 4.1

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8
Q

Hgb A

A

Adult hemoglobin

2 alpha, 2 beta globin chains

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9
Q

Cytoskeleton of red blood cell - vertical

A

Spectrin-ankryn-band 3

Spectrin-protein 4.1 - glycophorin

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10
Q

Hgb A2

A

2 alpha and 2 delta chains

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11
Q

Hgb F

A

Fetal hemoglobin

2 alpha and 2 gamma globin chains

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12
Q

Heme portion of hemoglobin

A

porphyrin ring and Fe2+

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13
Q

Oxygen dissociation curve

A

relates percent Hgb saturation to partial pressure of oxygen

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14
Q

Factors that influence oxygen binding

A
  • temperature
  • pH
  • organic phosphates
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15
Q

Anemia

A

a reduction in circulating erythrocyte mass

decreased:
hemoglobin
hematocrite
RBCs

*there will be decreased oxygen transport to tissues = tissue hypoxia

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16
Q

Deoxygenated blood has a _______ oxygen affinity.

A

Low

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17
Q

What might we see on examination of anemic patient?

A
  • tachycardia
  • SOB
  • systolic murmur
  • “severe signs” : syncope, signs of hypovolemia, tissue hypoxia, patient is very fatigued
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18
Q

How does the body compensate for anemia

A
  • increase cardiac output
  • increased respiratory rate
  • preferential blood flow to vital organs
  • decreased oxygen affinity
  • increased erythrocyte production
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19
Q

Microcytic

A

small size RBC

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20
Q

Normocytic

A

normal size RBC

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21
Q

Normochromic

A

normal amount of hemoglobin

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22
Q

Hypochromic

A

small amount of hemoglobin

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23
Q

Macrocytic

A

large RBC

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24
Q

Anemia from acute blood loss

A
  • Normocytic, normochromic anemia
  • volume depletion
  • decreased perfusion
  • extravascular fluid mobilization
  • recovery is slow as RBCs need to be manufactured for replacement
  • cells may have polychromasia during the recovery phase
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25
Q

Iron-deficiency anemia

A
  • interferes w/ normal Hgb

- impairs erythropoiesis

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26
Q

Hyperchromic

A

large amount of hemoglobin

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27
Q

Etiologic factors of iron-deficiency anemia

A
  • dietary deficiency (most common cause)
  • pregnancy/lactation
  • chronic blood loss
  • gynecological blood loss
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28
Q

Most common cause of anemia worldwide

A

iron-deficiency anemia

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29
Q

Transferrin

A

Delivers iron to cells including erythroid precursors

iron is transported in the plasma by transferrin

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30
Q

Total iron-binding capacity (TIBC)

A

indicates the maximum amount of iron needed to saturate plasma or serum transferrin

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31
Q

Ferritin

A

derived from the storage pool of iron and closely CORRELATES W/ TOTAL BODY IRON

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32
Q

If ferritin is high

A

lower binding capacity of iron

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33
Q

If ferritin is low

A

lots of capacity to bind iron

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34
Q

Iron-deficiency anemia (pathology)

A
  • microcytic, hypochromic anemia
  • anisopoiklocytosis (variation in erythrocyte size and shape)
  • no reticulocytes
  • serum iron decreased
  • ferritin decreased
  • transferrin and TIBC increased
  • transferrin iron saturation is low
  • RDW WILL ALWAYS BE LARGER, IN GENERAL
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35
Q

Iron deficiency anemia (appearance of cells)

A

area of pallor is bigger

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36
Q

Iron-deficiency anemia (treatment)

A
  • correct blood loss

- iron supplementation

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37
Q

Iron-deficiency anemia (clinical features)

A
  • Atrophic glossitis (smooth glistening tongue)
  • Angular stomatitis (inflammation at the corners of mouth)
  • Koilonychia (spoon-shaped deformity of fingernails)
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38
Q

Anemia of Chronic Disease

A
  • occurs w/ inflammatory or malignant conditions
  • mild to moderate anemia
  • normochromic, normocytic; can be microcytic
  • IRON STORES CAN BE NORMAL OR EVEN INCREASED
  • FUNCTIONAL IRON DEFICIENCY (body has enough iron, it’s just not able to use it)
  • decreased erythroid iron
  • decreased EPO
  • erythrocyte lifespan can be decreased
  • INFLAMMATORY CYTOKINES MAY DECREASE IRON MOBILIZATION
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39
Q

Aplastic Anemia

A

Disorder of pluripotent stem cells that lead to BONE MARROW FAILURE

  • ALL BLOOD ELEMENTS ARE DECREASED (PANCYTOPENIA)
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40
Q

Aplastic anemia - causes

A

insult to stem cells

  • idopathic (2/3 of cases)
  • drugs
  • chemicals
  • virsues
  • hereditary

dose dependent - w/ drugs and chemicals
idiosyncratic - dose independent, immunologic injury as see in idiopathic cases or after viral infections

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41
Q

Aplastic anemia (Pathology)

A
  • decreased bone marrow cellularity
    (Anemia, thrombocytopenia, and leukopenia; granulocytopenia is most common)
    Because of the precursors involved, they tend to have a DECENT AMOUNT OF LYMPHOCYTES
  • increased fat
  • EPO levels increase but there is no reticulocytosis
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42
Q

Anemia of Chronic Disease: TIBC, Ferritin, and Transferrin levels

A

Total iron binding capacity - DECREASES
Ferritin - normal, maybe a little increased
Transferrin - normal, sometimes decreased

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43
Q

Aplastic anemia (clinical presentation)

A

Pancytopenia effects including weakness, easily fatigued, bleeding, and infections
-Median survival 3-6 months

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44
Q

Pure Red Cell Aplasia (pathology)

A
  • Absence of erythroid precursors (the rest of bone marrow cellularity is normal)
  • EPO is decreased - NO RETICULOCYTES
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45
Q

Aplastic anemia (treatment)

A
  • immunosuppressive therapy

- bone marrow transplant

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46
Q

Pure Red Cell Aplasia

A

Selective suppression of committed red cell precursors in the bone marrow (white cells and platelets unaffected)

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47
Q

Parvovirus B19

A

Causes a relapsing form of Pure Red Cell Aplasia

  • viral inclusions in proerythroblasts
  • usually self limited
  • aplastic crisis when there is underlying anemia
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48
Q

Anemia of Renal Disease

A
  • Normocytic and normochromic

- DECREASED EPO in proportion to disease severity

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49
Q

Burr Cells

A

ANEMIA OF RENAL DISEASE

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50
Q

Schistocytes

A

If there is malignant hypertension w/ ANEMIA OF RENAL DISEASE, cells may be fragmented

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51
Q

Diamond-Blackfan Syndrome

A

PURE RED CELL APLASIA

  • seen in first 2 years of life
  • short stature, cleft lip or palate, micrognathia, limb abnormalities
  • defective erythroid precursors
  • poor EPO response
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52
Q

Myelophthisic Anemia

A 
Associated w/ BONE MARROW INFILTRATION
- myelofibrosis
- hematologic malignancies
- metastatic carcinomas
- granulomatous disease (competing for bone marrow space)
Normocytic Anemia
Leukoerythroblastosis
EXTRAMEDULLARY ERYTHROPOIESIS is a compensatory mechanism
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53
Q

Anemia of lead poisoning

A

Interferes w/ enzymes involved in heme synthesis

  • alpha-amnolvulinic acid dehydratase
  • Ferrochelatase
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54
Q

How do you expect cells to look on the peripheral smear for anemia of lead poisoning?

A

Microcytic

*often confused w/ iron deficiency anemia

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55
Q

Megaloblastic Anemia (physical features)

A
  • small nucleus
  • increased erythrocyte precursor pool (but cells are not as easily distributed in the bone marrow)
  • normal cytoplasm
  • nuclear-to-cytoplasm asynchrony
  • large precursor cells - MEGALOBLASTS
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56
Q

Megaloblastic Anemia

A

defective DNA synthesis secondary to B12 or folic acid (B9) deficiency

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57
Q

Megaloblastic anemia - from folate/folic acid deficiency

A
  • deficiency is usually inadequate dietary intake
  • alcoholics
  • pregnancy/lactation
  • inflammatory bowel disease (may interfere w/ absorption of folic acid)
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58
Q

Megaloblastic anemia - Pernicious anemia

A
  • autoimmune disorder
  • antibodies to parietal cells and intrinsic factor
  • previous surgery could cause injury
  • can decrease acid production (achlohydria)
  • fish tapeworm (Diphyllobothrium)
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59
Q

Megaloblastic anemia (pathology)

A
  • bone marrow shows increased hematopoietic activity w/ large precursors
  • Large RBCs result; anisopoikylocytosis
  • intermedullary cell death is increased
  • fewer cells of larger size
  • large bands form and hypersegmented neutrophills
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60
Q

B12 deficiency (clinical presentations)

A
  • demyelinates the posterior and lateral columns
  • sensory and motor deficits may occur

*this does not occur w/ folate deficiency

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61
Q

How long does it take to develop and B12 deficiency?

A

Years

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62
Q

Thalassemia

A

Congenital anemias that result from deficient synthesis of globin chain subunits of normal hemoglobins

  • globin subunit of interest is reduced or absnent
  • abnormal or unstable structures lead to RBC fragility and destruction
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63
Q

Thalassemia (epidemiology)

A
  • common in Mediterranean areas

- also seen in areas where malaria has been prevalent (possible malaria protection from heterozygous state)

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64
Q

How long does it take to develop and folate deficiency?

A

months

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65
Q

Cooley Anemia

A

HOMOZYGOUS B-THALASSEMIA

  • moderate to sever microcytic and hypochromic anemia
  • anisopoikylocytosis w/ basophilic stippling
  • unstable a4 tetramers precipitate in the cytoplasm of developing erythroid precursors
  • SOME A2 IS PRESENT IN ALL B-THALASSEMIA SINCE THE DELTA GENE IS UPREGULATED
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66
Q

B0 type in Cooley Anemia

A

Produces fetal hemoglobin and A2

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67
Q

A patient w/ Cooley anemia has an increased amount of Hgb F… what happens to O2 delivery?

A

O2 delivery will be DECREASED because of the anemia and because of the increased oxygen affinity of Hgb F
- this will lead to bone marrow hyperplasia (abnormal facial features)

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68
Q

B+ type in Cooley Anemia

A

produces some hemoglobin A and A2 is mildly increased

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69
Q

B-thalassemia

A

Transcription is entirely or partially suppressed due to a point mutation
B0 and B+ types

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70
Q

Heterozygous B-Thalasemia

A
  • patients typically asymptomatic
  • iron absorption is increased
  • minimal anisocytosis
  • target cells and basophilic stippling occurs
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71
Q

a-thalassemia

A

typically caused by gene deletions

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72
Q

Silent Carrier a-thalassemia

A
  • 1 gene missing
  • CAN MAKE HEMOGLOBIN NOMRALLY
  • no clinical problems, blood count usually normal or has mild decreases in the RBC mean corpuscular volume (MCV) and mean corpuscular hemoglobin (MCH)
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73
Q

a-thalassemia trait

A

2 genes missing

  • can make Hgb A
  • usually no clinical problems but often will have changes in their complete blood count including mild anemia, decreased RBC, MCV, and MCH
74
Q

Hgb H disease (Heinz bodies) a-thalassemia

A

3 genes missing

  • cannot make as much Hgb A
  • increase in TETRAMERS OF BETA HEMOGLOBIN
  • Hgb H has high binding capacity for oxygen
  • patient has moderate to sever anemia, low MCH and MCV, high reticulocyte counts
  • severity depends on how much hemoglobin A is made
75
Q

Homozygous (Bart’s hemoglobin) a-thalassemia

A
  • 4 genes missing; a-thalassemia major
  • CANNOT MAKE ANY HgB A AND PRODUCES BART’S HEMOGLOBIN TETRAMERS OF GAMMA HEMOGLOBIN CHAINS
  • causes Hydrops Fetalis
76
Q

Procoagulant complexes

A
  • Prothrombinase complex

- 2 X-ase complexes

77
Q

Anticoagulant Complex

A

Protein C-ase complex

78
Q

Hydrops Fetalis

A

Homozygous a-thalassemia w/ Bart hgb = incompatible w/ life

  • heart failure develops due to profound tissue hypoxia and fluid accumulation in various compartments (at least 2 compartments)
  • massive hepatosplenomegaly due to extramedullary hematopoiesis
79
Q

Prothrombinase Complex

A

Xa/Va/PL complex cleaves factor II>IIa

  • COMMON PATHWAY
  • Ca is required
80
Q

Protein C-ase complex

A

made of alpha-thrombin-thrombodulin in the endothelial membrane

Protein C w/ its cofactor protein S inactivate VIIa and Va

81
Q

Purpura Simplex

A
  • extravascular dysfunction
  • transient occurence in the deep dermis
  • enhanced by NSAIDs and other drugs
82
Q

X-ase Complexes

A

TF/VIIa/PL complex initiates coagulation

IXa/VIIIa/PL complex activates X

83
Q

Senile Purpura

A
  • extravascular dysfunction
  • older people; atrophy of supporting connective tissue
  • sharply demarcated persistent purpuric spots on forearms and other sun exposed areas
84
Q

Rendu-Osler-Weber Syndrome

A
  • vascular dysfunction
  • intrinsic genetic defects
  • AD disorder of vneules and capillaries
  • results from thinning of vessel wall due to inadequate elastic tissue and smooth muscle
85
Q

Scurvy

A
  • extravascular dysfunction
  • decreased Vitamin C disturbs collagen synthesis
  • perifollicular hemorrhages and purpura are seen
86
Q

Telangectasias of mucous membranes and dermis

A

Rendu-Osler-Weber Syndrome

87
Q

Arteriovenus malformations of solid organs

A

Rendu-Osler-Weber Syndrome

88
Q 
Mutation of TGF-Beta
also ENG (endoglin) and ALK1 (activin receptor-like kinase 1)
A

Rendu-Osler-Weber Syndrome

89
Q

Henoch-Schonlein Purpura

A
  • results from immunologic damage to the vessel wall
  • leukocytoclastic VASCULITIS
  • seen after viral illnesses in children
  • abdominal pain, arthralgia
  • renal involvement in 20-50% of cases
90
Q

Rendu-Osler-Weber Syndrome (clinical features)

A
  • begin as red colored punctate lesions
  • easy to bleed, develop some anemia
  • Recurrent epistaxis when younger (80%)
  • various organs can be involved and having varying degrees of pathology (shunting of blood since there is no buffering from capillary)
91
Q

PT

A

Prothrombin time

  • evaluates factors of EXTRINSIC AND COMMON PATHWAY
  • monitor COUMADIN
92
Q
  • Perivascular infiltration of neutrophils and eosinophils
  • fibrinoid necrosis of vessel walls
  • platelet plugs in the vasuclar lumen
  • IgA-compelement complexes found circulating and some in vessel walls
A

Henoch-Schonlein Purpura

93
Q

What does ITP stand for?

A

Idiopathic Thrombocytopenic Purpura

94
Q

PTT

A

Partial thromboplastin time

  • evaluates factors in the INTRINSIC AND COMMON PATHWAYS
  • monitor HEPARIN
95
Q

ITP

A
  • decreased platelets in the blood due to antibodies to platelet or megakaryocyte antigens (autoimmune)
96
Q

Peripheral smears show large platelets and bone marrow has increased megakaryocytes

A

ITP

97
Q

Acute ITP

A
  • usually in children
  • follows viral illness
  • platelet antigens attacked by autoantibodies
  • complement is bound
  • platelets are destroyed in the circulation (MAC attack) or phagocytized in the spleen or liver by macrophages
98
Q

IgG is detected on platelets in 80%; C3 is present on some

A

ITP

99
Q

Chronic ITP

A
  • usually in adults
  • more women affected
  • associated w/ collagen diseases, lymphoproliferative disease, and HIV
100
Q

Acute ITP (clinical features)

A
  • petechiae, purpura, epistaxis
  • intracranial hemorrhage
  • 80% spontaneously resolve within 6 months
  • glucocorticoids decrease antiplatelet antibody production and down-regulate Fc receptors on macs
  • gamma-globulin prevents clearing of IgG coated platelets
101
Q

Chronic ITP (clinical features)

A
  • bleeding episodes such as epistaxis, menorrhagia, or ecchymosis
  • treatment w/ glucocorticoids, Danazol, or gamma-globulin
  • if no response over time, splenectomy helps
102
Q

What does TTP stand for?

A

Thrombotic Thrombopenic Purpura

103
Q

TTP

A 
- Thrombotic microangiopathy
Pentad
- fever
- thrombocytopenia
- microangiopathic hemolytic anemia
- transient neurologic deficits 
- renal failure
104
Q

What is TTP due to?

A

excessive activation of platelets; there is deposition as thrombi in microcirculatory beds

105
Q

ADAMTS13 or VWF metalloprotease is deficient

  • decreased vWF cleavage of large multimers
  • greater chance of binding platlets
A

TTP

106
Q

PAS-positive hyaline microthrombin arterioles - diffusely located but especially seen in the brain, heart, and kidneys

A

TTP

107
Q

How is TTP different than vasculitis?

A

NO INFLAMMATION IN TTP

108
Q

What does HUS stand for?

A

Hemolytic uremic syndrome

109
Q

TTP clinical features

A
  • women during 4 or 5th decade of life
  • chronic and recurrent
  • weakness, seizures, aphasia, altered consciousness (stroke-like symptoms)
  • purpuric lesions
  • hemolytic anemia w/ decreased Hgb/Hct
  • Jaundice
  • Renal insufficiency may develop
  • PT and PTT are normal
110
Q

Follows gastroenteritis caused by E.coli 0157:H7 or Shigella dysenteriae

Kidney failure main clinical feature

A

HUS

111
Q

Kasabach-Merritt Syndrome

A
  • a cause of thrombocytopenia (tumors sequester platelets)

- baby pictures

112
Q

TTP pathology

A
  • Schistocytes (fragmented erythrocytes) are seen on peripheral blood smears due to shear force of microthrombi
  • Bilirubin would be elevated because we’re breaking down RBCs
113
Q

Bernard-Soulier Syndrome

A
  • AR platelet disorder
  • GIANT PLATELETS
  • GpIb does not attach to vWF
  • patients present w/ petechiae, ecchymosis, epistaxis, bleeding gingiva; often in children
  • can present as GI bleeding or menorrhagia as adults
114
Q

Hemophlia A (clinical features)

A

mild to severe bleeding tendencies

  • 1/2 have virtually no activity (spontaneously bleed)
  • may bleed into joints and have secondary degeneration
  • intracranial bleed
  • hematuria, intestinal obstruction and respiratory obstruction can occur due to bleeding in these organs
115
Q

Hemophilia B

A

X linked disorder of deficient Factor IX (9)

  • Factor IX made in the liver and is vitamin K dependent
  • variety of mutations are causative
  • manifestations are similar to Hemophilia A
116
Q

Hemophilia A

A

Most common X-linked inherited bleeding disorder

Factor VIII (8) on Xq28 can have deletions, insertions, point mutations, and inversions

117
Q

Hemophilia A (treatment)

A

replacement of the factor

118
Q

von Willebrand Disease

A
  • deficiency or abnormality of vWF

- most common INHERITED bleeding disorder of humans

119
Q

Hemophilia B (treatment)

A

replacement of factor IX

120
Q

vWF is produced by…

A

endothelial cells and megakaryocytes

121
Q

vWF gene is located on chromosome…

A

Chromosome 12

122
Q

Weibel-Palade bodies

A

where vWF is stored (vWF is adhesive)

123
Q

Type I vWD

A

75% of disease

AD inheritance, variable penetrance
quantitative deficiency (levels of all multimers are reduced)
124
Q

Type II vWD

A
  • 20% of disease
  • AD inheritance
  • activities of vWF and factor VIII are decreased
125
Q

Type IIa VWD

A

ABSENCE of large multimers

126
Q

Type IIb vWD

A

abnormally high PLATELET AFFINITY

127
Q

vWD (clinical features)

A
  • mild bleeding that goes unnoticed until there is some type of hemostatic stress such as a medical/dental procedure (type III is the exception)
128
Q

Type III vWD

A
  • AR inheritance; compound heterozygotes (mutant alleles on each chromosome)
  • Most severe and least common
  • vWF is ABSENT and Factor VIII is less than 10% of normal
129
Q

vWD (treatment for I and II)

A

DDAVP, causes endothelial release of vWF

130
Q

vWD (treatment for III)

A

vWF and/or cryoprecipitate

131
Q

What factors does the liver produce?

A

2, 5, 7, 9, 10

132
Q

What can be prolonged as a result of liver disease and coagulopathies?

A

PT and PTT; PT more severely affected since factor 7 is the workhorse

133
Q

Which factor is not vitamin K dependent?

A

5

134
Q

Decrease in Vitamin K

A

process of y-carboxylation in factors 2, 7, 9, and 10 is decreased

135
Q

DIC

A
  • A thrombohemorrhagic disorder characterized by excessive activation of coagulation that leads to thrombi formation in the microvasculature (arterioles, venules, capillaries)
  • can be widespread
  • coagulation factors are consumed
  • NOT A PRIMARY DISEASE, caused by a variety of clinical disorders
  • ischemia ensues
136
Q

What does DIC stand for?

A

Disseminated Intravascular Coagulation

137
Q

Treatment of DIC

A

heparin
platelets
clotting factors
treating the underlying disease

138
Q

Most likely associations w/ DIC

A

malignant neoplasms
sepsis
obstretric complications
major trauma or burns

139
Q

Neutrophilia

A

ANC > 7000

  • caused by mobilization from the bone marrow
  • enhanced release from the peripheral blood marginal pool
  • increased granulopoiesis
140
Q

Agranulocytosis

A

virtual absence of neutrophils

141
Q

Neutropenia

A

Absolute neutrophil count (ANC) < 1500

142
Q

Eosinophilia

A
  • eosinophils differentiate in the bone marrow
  • migrate to skin, lungs, and GI tract
  • respond to chemotactic substances produced by mast cells or Ag-Ab complexes
143
Q

Leukemoid reaction

A
  • occurs in neutrophilia (cells are segmented neutrophils)
  • WBC < 50,000
  • Dohle bodies and toxic granulation
144
Q

Idopathic Hypereosinophilic Syndrome

A
  • eosinophils above 1500 for 6 months

- diagnosis also requires organ involvement and know identifiable cause for the eosinophilia

145
Q

Endomyocardial fibrosis

A

Idiopathic Hypereosinophilic Syndrome

146
Q

Idiopathic Hypereosinophilic Syndrome (clinical features)

A
  • Tissue necrosis (especially in heart)
  • Neurologic dysfunction
  • high mortality
147
Q

Loeffler’s endocarditis

A

Idiopathic Hypereosinophilic Syndrome

148
Q

Basophilia is typically due to…

A
  • immediate-type hypersensitivity reactions

- chronic myeloproliferative neoplasms

149
Q

Monocytosis

A
  • Monocyte count > 800
  • Due to infection, hematologic malignancies, solid cancers, immunologic conditions
  • monocytosis in a neutropenic state is likely compensatory
150
Q

Basophilia

A
  • least abundant leukocyte
  • differentiates in bone marrow
  • contains inflammatory mediators
151
Q

Myeloproliferative neoplasms

A

clonal hematopoietic stem cell disorders that cause PROLIFERATION OF ONE OR MORE MYELOID LINES

152
Q

Myelodysplastic syndromes

A

MARROW FAILURE, DYSPLASTIC CHANGES OF ONE OR MORE CELL LINES, and PERIPHERAL CYTOPENIA

153
Q

Reactive Mast Cell Hyperplasia

A
  • Not malignant
  • seen in lymph nodes that drain sites of malignant tumors
  • seen in immediate and delayed-type hypersensitivities
154
Q

Chronic Phase of CML

A
  • increased neutrophils in ALL stages of development
  • basophilia and eosinophilia
  • hypercellular marrow w/ abnormal precursors
155
Q

Most common myeloproliferative disease

A

CML

156
Q

What does CML stand for?

A

Chronic Myelogenous Leukemia

157
Q

What is necessary for the diagnosis of CML?

A

Philadelphia chromosome or bcr/abl fusion genes

- Tyrosine kinase activity of fusion protein p210 can induce proliferation, differentiation, and overall survival

158
Q

Association/cause of CML

A

radiation and benzene exposure - slight male predominance

159
Q

CML is derived from…

A

an abnormal bone marrow stem cell and results in prominent neutrophilic leukocytosis

160
Q

Accelerated phase of CML

A
  • increasing WBC
  • increasing splenomegaly
  • 10-20% blasts in the marrow or peripheral blood
  • thrombocytopenia/thrombocytosis
  • additional chromosomal abnormalities
161
Q

CML treatment

A
  • Imatnib competes for ATP-binding site on bcr/abl (tyrosine kinase won’t be able to be made)
  • bone marrow transplant can be used
162
Q

Polycythemia Vera (Major Criteria)

A

Hbg > 18.5 g/dL in men
Hbg > 16.5 g/dl in women
JAK 2 mutation

163
Q

Blast phase of CML

A
  • true CML

- 20% blasts in blood and/or marrow w/ EXTRAMEDULLARY PROLIFERATION OF THE BLASTS

164
Q

Polycythemia Vera (clinical features)

A
  • 60 years
  • insidious onset
  • splenomegaly is typical and found early
  • Circulatory problems/sluggish flow (blood is very thick)
  • hypoxia to various organs; angina, claudication
  • headache, dizziness, visual disturbances
165
Q

Polycythemia Vera minor critieria

A
  • NO EPO ELEVATION
  • Hypercellular marrow w/ panmyelosis
  • Erythroid colony formation in vitor w/o growth factor stimulation
166
Q

Polycythemia Vera

A
  • Myeloproliferative neoplasm arising from a clonal hematopoietic stem cell
  • Autonomous RBC production NOT UNDER THE CONTROL OF EPO
167
Q

Prepolycythemic phase

A

Borderline or mild erythrocytosis

168
Q

Polycythemic phase

A

defining erythrocytosis

169
Q

What occurs to patients w/ polycythemia vera perhaps due to chemotherapeutic agents used for treatment?

A

AML

170
Q

Postpolycythemic phase/spent phase

A
  • excessive proliferation stops
  • can become anemic
  • myelofibrosis develops
    (bone marrow has been spent)
171
Q

What does AML stand for?

A

Acute Myeloid Leukemia

172
Q

Myelodysplastic Syndromes

A
  • cytopenia of the peripheral blood
  • bone marrow failure
  • dysplastic morphology of 1 or more cell lines

discrepancy between amount of peripheral blood elements and marked hyperplasia seen in the bone marrow

173
Q

Essential Thrombocythemia

A
  • uncommon neoplastic disorder of hematopoietic stem cells
  • uncontrolled proliferation of megakaryocytes (increased number of thrombocytes)
  • Platelets > 600,000
  • Thrombosis and hemorrhage can be recurrent
174
Q

What is a major difference between Myelodysplastic syndromes and Myelodysplastic neoplasms?

A

In Myelodysplastic syndromes, there is not erythrocytosis, leukocytosis, or thrombocytosis as seen in the MPN diseases

175
Q

Why are myelodysplastic syndromes paradoxical

A

Bone marrow remains hypercellular but there is hematopoeisis and apoptosis

176
Q

What is the major problem w/ AML?

A

Accumulation of immature and undifferentiated myeloid cells in the bone marrow

177
Q

Auer rods

A

AML

178
Q

Acute suppurative lymphadenitis - posterior auricular

A

rubella

179
Q

Acute suppurative lymphadenitis - occipital nodes

A

scalp infections

180
Q

AML

A
  • clonal proliferation of myeloblasts in the bone marrow followed by appearance in the blood and possible in tissues
  • diagnosis requires MORE THAN 20% MYELOBLASTS IN THE PERIPHERAL BLOOD OR BONE MARROW
  • bone marrow is infiltrated and displaced w/ malignant cells
181
Q

Acute suppurative lymphadenitis - axillary

A

infection of upper extremity

182
Q

Acute suppurative lymphadenitis - posterior cervical

A

toxoplasmosis

183
Q

Acute suppurative lymphadenitis - generalized

A

systemic infections

Pathophysiology (4 decks)

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