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Haematology > Myeloproliferative Neoplasms > Flashcards

Myeloproliferative Neoplasms Flashcards

1
Q

are myeloproliferative disorders and neoplasms the same thing

A

disorders old term, neoplasms new term

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2
Q

what is the difference between polycythaemia rubra vera and PV

A

PRV old term

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3
Q

what does ‘myelo’ mean

A

bone marrow lineage- granulocyte, red cells, platelets

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4
Q

what are myeloproliferative neoplasms

A

clonal haemopoietic stem cell disorders with increased production of one/ more types of haemopietic cells

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5
Q

what is maturation of cells like in MPN

A

preserved (unlike acute leukaemias)

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6
Q

what are the subtyprs of MPN

A

BCR-ABL1 +ve
-CML (overproduction of granulocytes, philadelphia chromosome)

BCR-ABL1 -ve

  • essential thrombocythaemia (over production of platelets)
  • primary myelofibrosis
  • polycythaemia vera (overproduction of red cells)
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7
Q

when should you consider MPN as a diagnosis IMPORTANT

A 
when there is no reactive explanation for: 
high granulocyte count 
\+/-
high red cell/ Hb 
\+/-
high platelet count 
\+/-
eosinophilia/ basophilia

splenomegaly
thrombosis in an unusual place

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8
Q

what are the features of chronic myeloid leukaemia

A

proliferation of myeloid cells:

  • granulocytes + precursors
  • other lineages (platelets)

chronic phase with intact maturation followed by an accelerated phase and finally a blast crisis with maturation defect

fatal without stem cell/ bone marrow transplant in the chronic phase

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9
Q

what are the clinical features of CML

A

asymptomatic
splenomegaly
hypermetabolic symptoms
gout

problems due to hyperleucocytosis: priapism

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10
Q

what are the lab features of CML

A

normal/ low Hb
leucocytosis with neutrophilia and myeloid precursors (myelocytes), eosinophilia, basophilia
thrombocytosis

(compared to reactive will have higher WBC, neutrophils, eosinophils and basophils. Will have lower monocytes and lymphocytes)

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11
Q

what is the hallmark of CML

A

the philadelphia chromosome

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12
Q

what is BCR-ABL1

A

the chiameric gene that results from the philadelphia chromosome (CML)

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13
Q

what is the gene product of BCR-ABL1

A

tyrosine kinase

this causes abnormal phosphorylation = haematological change in CML

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14
Q

what drugs work in CML

A

tyrosin kinase inhibitors e.g. Imatinib

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15
Q

what features are common to all MPN

A

Asymptomatic

Increased cellular turnover (gout, fatigue, weight loss, sweats)

Symptoms/signs due to splenomegaly

Marrow failure (fibrosis or leukaemic transformation:lower with PRV and ET)

Thrombosis (arterial or venous including TIA, MI, abdominal vessel thrombosis, claudication, erythromelalgia)

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16
Q

what is polycythaemia vera

A

high Hb/ haematocrit accompanied by erythrocytosis (a true increase in red cell mass)
can have excessive production of other lineages

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17
Q

what must you differentiate PV from IMPORTANT

A

secondary polcythaemia (chronic hypoxia, smoking, erythropoietin secreting tumour)

pseudopolycythaemia (dehydration, diuretic therapy, obesity)

18
Q

what are the clinical features specific to PV

A

headache
fatigue
itch (aquagenic puritis)

19
Q

what viscosity is raised in PV

A

blood viscosity NOT plasma viscosity (why you get HAs)

20
Q

how do you investigate polcythaemia

A 
Hx (exclude secondary polycythaemia) 
exam (splenomegaly?)
FBC
blood film 
JAK2 MUTATION STATUS IMPORTANT (JAK2 analysis)

look for secondary/pseudo causes: CXR, O2 sats/ ABGs. drug Hx

infrequent tests: erythropoietin levels, bone marrow biopsy

21
Q

what is JAK2

A

a kinase

22
Q

how many of patients with PV have a JAK2 mutation

A

95%

23
Q

what does a JAK2 mutation (substitution) cause

A

loss of auto-inhibition

activation of erythropoiesis in the absence of a ligand

24
Q

what test forms part of the initial screening for PV

A

JAK2 mutation analysis

25
Q

what is the treatment for PV

A

venesect to haematocrit <0.45
aspirin
cytotoxic oral chemo (hydroxycarbamide)

26
Q

what is essential thrmobocythaemia

A

uncontrolled production of abnormal platelets

causes abnormal platelet function:

  • thombosis
  • at high levels causes bleeding due to acquired VWF disease
27
Q

what are the clinical features specific to ET

A

bleeding

vasoocclusive complications

28
Q

how does ET compare to reactive thrombocytosis

A

Hb, platelets, RBCs, haematocrit, eosinophils and basophils higher
wbc and neutrophils lower

29
Q

how is ET diagnosed

A

EXCLUDE REACTIVE THROMBOCYTOSIS (blood loss, inflammation, malignancy, iron deficiency)

exclude CML

JAK2 mutation in 50-60%, CALR in 25%, MPL in 5%, 10-20% triple negative

characteristic bone marrow appearances

30
Q

what is the treatment for ET

A

anti-platelets (aspirin)

cytoreductive therapy: hydroxycarbamide, anafrelide, interferon alpha

31
Q

what is myelofibrosis

A

hyperplasia of megakaryocytes which produce platelet-derived growth factor= intense marrow fibrosis and haematopoiesisin the spleen and liver

32
Q

what causes myelofibrosis

A

idiopathic

post-polycythmaeia/ essential thrombocythaemia

33
Q

what are the features of idiopathic myelofibrosis

A

marrow failure
bone marrow fibrosis
extramedullary haematopoiesis (liver and spleen)
LEUKOERYTHROBLASTIC FILM APPEARANCES IMPORTANT
tearsrop shaped RBCs in peripheral blood

34
Q

what are the clinical features of myelofibrosis

A

marrow failure: anaemia, bleeding, infection

splenomegaly (LUQ pain, comps e.g. portal hypertension)

hypercatabolism

35
Q

how is myelofibrosis diagnosed

A

blood film: tear drop shaped RBCs and leucoerythoblastic

dry aspirate

fibrosis on trephine biopsy

JAK2, CALR, MPL mutations

36
Q

what can cause a leucoerythroblastic film IMPORTANT

A

(immature cells and teardrop RBCs)
reactive (sepsis)
marrow infiltration
myelofibrosis

37
Q

what is the treatment for myelofibrosis

A 
supportive care (blood transfusion, platelets, antibiotics)
allogenic stem cell transplantation (select few patients)
splenectomy (controversial) 
JAK2 inhibitors (improve spleen size and constitutional symptoms)
38
Q

are reactive causes or MPN more common

A

reactive causes

39
Q

summaries the reactive causes resulting in increased cells

A

Granulocytes

  • Infection: eg pyogenic bacteria causing neutrophilia
  • Physiological eg post-surgery, steroids

Platelets

  • Infection
  • Iron deficiency
  • Malignancy
  • Blood loss

Red cells

  • Dehydration (diuretics): pseudopolycythaemia
  • Secondary polycythaemia (eg hypoxia-induced)
40
Q

how can you tell the difference between reactive changes an MPN

A

Hx and exam

reactive changes unlikely to be associated with the comps of MPN

Haematology (27 decks)

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