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HEMATOLOGY > Myeloproliferative Disorders > Flashcards

Myeloproliferative Disorders Flashcards

1
Q

What are MPN?

A

clonal haemopoietic stem cell disorders with an INCR. prodn of ONE or MORE types of haemopoietic cells

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2
Q

How is MPN diff. from acute leukaemia?

A
  • maturation is relatively preserved in MPN
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3
Q

What is BCR-ABL 1?

A
  • a when ABL gene from Chr. 9 fuses with the BCR gene on chromosome 22
  • it form a fusion gene on chromosome 22; known as the Philadelphia Chromosome
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4
Q

Who carries the Philadelphia chromsome?

A
  • those who have CHRONIC MYELOID LEUKAEMIA
  • —also seen in Acute Lymphoblastic Leukaemia and Acute myeloid leukaemia
  • “BCR-ABL1 + “
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5
Q

Are MPN BCR-ABL 1 +?

A

NO

- they are philadelphia chr. -

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6
Q

Name BCR- ABL 1 -ve disorders.

A
    1. primary myelofibrosis
      1. Polycythemia vera
      2. essential thrombocytopenia
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7
Q

When do you suspect MPN?

A
  1. high granulocyte count
  2. High RED CELL count/ Hb
  3. High PLATELET counts
  4. Eosinophilia and Basophilia
  5. SPLENOMEGALY
  6. Thrombosis in an unusual place
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8
Q

What is CML?

A
  • chronic myeloid leukaemia

- —proliferation of MYELOID cells ! (granulocytes and their precursors) & other lineages (platelets)

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9
Q

How does CML progress over the years?

A
  1. initial CHRONIC phase with INTACT maturation for 3-5 years (slow progression)
  2. half of CML cases enter accelerated phase
  3. final transformation into condition similar to ACUTE leukaemia
    - –followed by blasts crisis (maturation defect)
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10
Q

CML is fatal without _________

A

stem cell/ bone marrow transplant !

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11
Q

What are the clinical fts of CML?

A
  1. asymptomatic ; nonspecific ( FATIGUE/ weak/ WGT LOSS)
  2. 1st sx: DRAGGING sensation (splenomegaly)
  3. hypermetabolic symptoms
  4. miscel.: problems related to hyperleucocytosis, priapism
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12
Q

What occurs in the accelerated phase of CML?

A
  • marked ANAEMIA

- new thrombocytopenia

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13
Q

What are some blood count changes seen with CML?

A
  1. NORMAL/ decr. Hb
  2. leucocytosis with Neutrophilia and myeloid precursors, eosinophila, basophilia
  3. thrombocytosis
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14
Q

So how does the Philadelphia chromosome bring about the hematological changes in CML?

A
  • the gene product that results from the genetic changes is TYROSINE KINASE

—-> causes abnormal phosphorylation (signalling) leading to CML

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15
Q

What can be given to treat CML?

A

IMATINIB

—–d.t response with this tyrosine kinase INHIBITOR

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16
Q

What are fts common to MPN?

A
  • incr. cellular turnover (wgt loss/ gout/ fatigue/sweats )
  • signs/sx d.t splenomegaly
  • marrow failure (FIBROSIS/ leukaemic transformation: lower with PRV and ET)
  • thrombosis (arterial/ venous- TIA/ MI/ abdominal thrombosis/ claudication/ erthromyelgia)
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17
Q

What is Polycythaemia vera?

A
  • high Hb/ Hematocrit accompanied by erythrocytosis (true incr. in red cell mass)
  • excessive proliferation of erythroid, granulocytic and megakaryocytic elements
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18
Q

Polycythaemia Vera should be distinguished from?

A
  1. IIary Polycythaemia (chronic hypoxia/ smoking/ erythropoietin-secreting TUMOR)
  2. Pseudopolycythaemia (dehydration/ obesity/ diuretic therapy)
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19
Q

How to tell pseudopolycythaemia from True polycythaemia?

A
  • TRUE: actually has an INCR. RED CELL MASS
20
Q

Clinical Fts of PV?

A
  1. headache/ fatigue (BLOOD viscocity raised- not plasma)

2. ITCH

21
Q

What ivx could be done for polycythaemia?

A
  • hx ( r.o IIary and pseudo-)
  • examination
  • FBC/ FILM
  • JAK2 mutation status!
22
Q

What particular genetic mutation is seen in 95% of PV?

A
  • JAK2 mutation
    —a kinase; the mutation results in LOSS of AUTO-INHIBITION
    > activation of erythropoiesis in the ABSENCE of A ligand
23
Q

What should be done during the INITIAL screening of PCV?

A
  • mutational ANALYSIS!

- –it has replaced a no, of other tests in routine practise!

24
Q

What are the ivx for PCV?

A
  1. ivx to R.O secondary/pseudo cause: CXR, O2 sat., arterial blood gases, drug hx)
  2. JAK2 analysis
  3. infreq. tests: erythrpoietin levels, bone marrow biopsy
25
Q

Name some erythropoietin secreting tumors.

A
  1. Renal cell carcinoma
  2. Hepatocellular CA
  3. Adrenal Adenoma
  4. Uterine tumors
26
Q

How to treat PV?

A
  • venesection until haematocrit <0.45
  • aspirin
  • cytotoxic oral chemo (hydroxycarbamide)
27
Q

What is essential Thrombocytopenia?

A
  • uncontrolled prodn of ABNORMAL platelets!

- platelet fxn that is abnormal (==> thrombosis/ at HIGH levels may cause BLEEDING due to acquired vWF disease)

28
Q

What are the clinical fts of ET?

A
  • common to MPN (esp. vasoocclusive complications)

- bleeding (unpredictable risk esp. at surgery )

29
Q

How to come to the dx of ET?

A
  1. EXCLUDE reactive thrombocytosis (blood loss/ inflammation/ malignancy/ iron def.)
  2. exclude CML
  3. characteristic marrow fts
  4. genetics test
30
Q

What genetic changes are seen in ET?

A
  • JAK2 in 50-60%
  • MPL (5%)
  • -CALR (in approx. 25%)
  • 10-20% of pts will be TRIPLE NEGATIVE
31
Q

How to treat ET ?

A
  • anti-platelet agents: ASPIRIN

- cytoreductive therapy to control proliferation (hydroxycarbamide/ anagrelide/ interferon alpha)

32
Q

When may myelofibrosis occur?

A

after Essential Thrombocythaemia

33
Q

What are the clinical fts of myelofibrosis?

A
  1. marrow failure (anaemia/ bleeding/ infection)
  2. splenomegaly
    - LUQ pain
    - portal hypertension
  3. hypercatabolism
34
Q

What occurs pathologically in the course of idopathic myelofibrosis?

A
  1. MARROW FAILURE (variable degrees)

> bone marrow fibrosis

> haematopoiesis shifts to the LIVER, spleen and lymph nodes

35
Q

How does idiopathic myelofibrosis, appear on film?

A
  • LEUKOERYTHROBLASTIC film appearances
  • TEARDROP-shaped RBCs in peripheral blood
  • fibrosis on trephine biopsy
36
Q

What mutations are seen in MF?

A
  • JAK2 > CALR> MPL

- —10% is triple negative

37
Q

When may leukoerythroblastic film appear?

A
  • reactive (sepsis)
  • marrow infiltration
  • myelofibrosis
38
Q

What is meant by leukoerythroblastic film?

A
  • the presence of IMMATURE cells (normoblasts/ myelocytes) seen in the peripheral blood film
  • norochromic, normocytic anaemia =- numerous poikilocytes
  • low gr. reticulocytosis
  • immature WHITE cells (myelocytes and promyelocytes)
  • thrombocytopenia > thrombocythaemia
39
Q

Thrombocythaemia vs thrombocytosis?

A
  • thrombocythaemia = when the elevated platelet count is d.t an UNKNOWN cause !
40
Q

How to treat myelofibrosis?

A
  • supportive care = blood transfusion/ platelets/ antibiotics
  • allogenic stem cell transplantation
  • splenectomy
  • JAK2 inhibitors (improves spleen size/ constitutiona symptoms)
41
Q

Name reactive causes of granulocytosis.

What is this form of reactiton called and what should this be distinguished from?

A
  1. infections: pyogenic bacteria (neutrophilia!)
  2. physiological (post-surgery; surgery; steroids )
  • leukamoid reaction; diff. from CML
42
Q

What are reactive causes of platelets?

A
  • infection
  • iron def.
  • malignancy
  • blood loss
43
Q

Reactive causes of high RED cell ounts?

A
  • dehydration (diuretics)–> PSEUFOPOLYCYTHAEMIA

- secondary polycythaemia (hypoxia induced)

44
Q

How to distinguish leukamoid rxn from CML?

A

-testing for presence BCR-ABL fusion gene —done by PCR assay/ karyotyping

45
Q

How to confirm is it’s a leukamoid reaction?

A
  • ELEVATED LAP score

leukocyte alkaline phosphatase

HEMATOLOGY (20 decks)

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