Myeloproliferative Disorder Flashcards

1
Q

Myeloproliferative syndrome defination ??

A
  • proliferative neoplastic disorder
  • uncontrolled proliferation of abnormal progenitor cells
  • which are capable of producing myeloid,erythroid,megakaryocytic series
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2
Q

names of myeloproliferative disease ?

A
  1. polycythemia rubra vera
  2. CML
  3. myelofibrosis
  4. essential thrombocythemia
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3
Q

what is myelofibrosis ??

A

fibrosis & collagen formation in the marrow

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4
Q

CBC of myelofibrosis ?

A
  1. Hb% -
  2. PCV -
  3. RBC -
  4. WBC-
  5. P -
  6. ESR +
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5
Q

PBF of Myelofibrosis ?

A
  1. RBC = N-N-A-P-tear drop cells - rouleux formation
  2. WBC -
  3. P -
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6
Q

BM of myelofibrosis ?

A
  1. C Acellular
  2. M/E Normal
  3. E -
  4. G-
  5. M-
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7
Q

other test for myelofibrosis ?

A
  • folic acid -
  • uric acid +
  • leukocyte alkaline phosphatse score +
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8
Q

Polycythemia-Def ?

A

elevated levels of all blood components

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9
Q

when it is True Polycythemia ?

A

Elevation in all blood count

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10
Q

types of True polycythemia ?

A
  1. P rubra vera
  2. secondary P
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11
Q

when it is relative Polycythemia ?

A

reduction in the plasma volume

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12
Q

causes of pseudo-P ?

A
  • stress
  • cigarette
  • burns
  • dehydration
  • reduced fluid intkae
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13
Q

CBC of Polycythemia Rubra Vera ?

A
  1. Hb% +
  2. PCV +
  3. RBC +
  4. WBC +
  5. P +
  6. ESR -
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14
Q

BM of PRV ?

A
  1. C= +
  2. M/E = Normal
  3. E - ERYTHROID hyperplasia
  4. G - HYPERACTIVE
  5. M -Hyperactive
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15
Q

Others test for PRV ??

A
    1. janus associated kinase- 2
  • loss/gain of C-9
  • NAP +
  • serum erythropoietin -
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16
Q

Causes of PRV ?

A
  1. Hypoxia
  2. high altitude
  3. heart disease
  4. heeavy cigarette smoker
  5. high affinity to Hb
  6. polycystic kidney disease
  7. renal d
  8. carcinoma
  9. beningn familial polycythemia
  10. 2,3 biphosphoglycerate deficiency
17
Q

Diagnosis Triad of PRV ??

A
  1. Splenomegaly
  2. elevated Hb with leukocytosis & thrombopcytosis
  3. dusky brick red colour of the face = Ruddy cyanosis
18
Q

WHO criteria major for PRV ?

A
  1. JAK-2 mutation
  2. Hb% male >18gm/dl
  3. female >16gm/dl
19
Q

minor criteria for PRV ?

A
  1. erythropoitin -
  2. inc bone marrow proliferation
20
Q

ss of PRV ?

A
  1. thrombosis
  2. DVT
  3. HTN
  4. PVD
  5. Hemorrhage
  6. hepatosplenomegaly
  7. headache
  8. dizziness
  9. pruritis
  10. poor vision
21
Q
A
22
Q
A