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Malignant haematology > Myelofibrpsos > Flashcards

Myelofibrpsos Flashcards

1
Q

What is myelofibrosis

A

Bone marrow is replaced with collagedn and become fibrosed

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2
Q

Aetiolofy of myelpfibrosis

A

Disease typically manifests >50, can occur in children
Familial relationship, preponderance in ashkenazi jews

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3
Q

Pathology myelofribrosis

A

Haematopoietic cell progenitor mutation in megakaryocute line -< proliferation of fibroblasts and collagen deposition reactive to cytokine production incl TGF-B
EPO impaired -> anaemia
Extramedullary haemtopoiesis

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4
Q

TGF-beta effects

A

Fibrosis
Osteoclast proliferation-> osteosclerosis

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5
Q

Clinical features of myelofibrosis

A

20% asymptomatic
Severe fatigue
Hepatosplenomegaly
Can get massive splenomegaly
B symptoms - weight loss, fever, night sweats
Anaemia
VTE secondary to thrombocytosis
unexplained bleeding -> TP
Sev bony pain - periosteal infalmmation and osteosclorosis

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6
Q

Extramedaullry haematopoiesis causing?

A

Seizures, paralysis, ascite, pericardial, abdominal, pleural effusions, raised ICP, lymphadenopathy
liver and spleen most common sites

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7
Q

Initial investigation myelofirbsosi

A

FBC, U+Es, LFTs, coag studies
Blood smear
LDH
Bone marrow exam
Gentic testing

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8
Q

Bloods in myelofibrosis

A

Pancytopenia
Decreased RBC
WCC and platelet - VARY
PT + APTT prolonged, raised ALP - hepatic involevemnt
LDH raised - RCC lysis

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9
Q

Blood film in myelofibrosis shows

A

Teardrop RBC - dacrocytes

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10
Q

How can examine bone marrow

A

Need a biopsy
Can do aspirate/MRI also
MRI monitor progression

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11
Q

Myelofibrosis - bone marrow aspirate what see

A

May yield dry tap
Success - entire myeloid lineage abnormal

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12
Q

Bone marrow biopsy what see in myelofibrss

A

Fibrosis and abnormal megakaryocyte appearance

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13
Q

MRI myelofibrosis what see

A

Decreased signal from bone marrow as fat replaced w fibrosis

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14
Q

Genetic ass w myelofibrosis

A

JAK2 -60%
CALR - 25%
MPL - 5%
Triple negative variant - 10%

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15
Q

Major criteria for myelofibrosis

A

Proliferation and atypia of megakaryocytes acc by fibrosis
Doesnt mathch other myeloid neoplasms
JAK2, CALR, MPL mutation
If absent mutations - clonal arker or absence of reactive myelofibrosis

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16
Q

Minor criteria for Myelofirbsois

A

Anaemia UEO
Leukocytosis >11 x10
Palpable splenomegaly
Raised LDH
LEUKOERYTHRoblastosis

17
Q

Non haematological causes of myelofibrosis

A

hyperparathyroidsim
SLE
vit D deficiency
Systemic sclerosis

18
Q

Treatment for asymptomatic patients myelofibrosis

A

none - routine follow up

19
Q

Curative treatment for myelofibrosis

A

ONly cure is haemtopoietic stem cell transplant

20
Q

Symptomatic/palleative treatment for myelofibrosis

A

Ruxolitinib - JAK2 inhibitor
Splenectomy, splenic irradiation if causing pain
Blood transfusions for anaemia or EPO injections
Extrameduallyr Haematpoiesis - foci irradiates
Aspirin - VTE
Hyperuricaemia - allopurinol

21
Q

How treat anaemia in myelofibrosis

A

Blood transusions - thalidomide and prednisone as adjuncts
Iron, B9, B12 deficiencies investigated and managed
EPO not shown to benefit

22
Q

Ruxolitibinib how works in myelofibrosis

A

Improves splenomegaly and constitutional symptoms
Works irrelevant of whether have JAK2 mutation
Effective regardless of whether splenomegaly present
Doesnt prolong survival

23
Q

Alternatives to ruxolitibin

A

Hydroxyurea , interferon alpha

24
Q

Most deadly complication of myelofibrosis

A

Progression to acute myeloid leukaemia

25
Q

Complications of myelofibrosis

A

Haemorrhage - thrombocytopenia
Infections - low WCC
Portal HPTN - hepatomegaly
Splenic infarctions
Sequelae raised urea eg gout, kidney stones
Neurologic manifestations - EMHS - seizures, cord compression, raised ICP
VTE earlier phases
Osteosclerosis

26
Q

Prognosis myelofibrosis

A

mean - 6 years
Massive variation
Most common cause death - AML
Others - infection, haemorrhage, thrombosis, cachexia

27
Q

What can turn into myelofibrosis

A

essentiall thrombocytopenia

28
Q

What need to review in essential thrombocytopenia

A

Cardiovacular risk factors - cholesterol, BP etc
Aspirin for all

29
Q

When do you ive cytoreduction in essential thrombocytoprnia

A

High risk eg
Over 60
Platelets >1500x 10^9/L
Prev thrombotic event
Other CVS risk factors

30
Q

cytoreduction treatment

A

Hydroxycarbamide - reduce throbotic events
Interferon, anagrelide
Busulfan

31
Q

What is cytoreduction treatment

A

Reducing platelets

32
Q

Presentation of essential thrombocytosis

A

Migraine
Erythromyalgia - burning pain
Itch
arterial and venous thrombosis
bleeding

33
Q

how confirm essential thromboytopenia

A

bone marrow biopsy

34
Q

Myelofibrosis vs essential thrombocytopenia

A

More agressive
Is teh proliferation of mutliple cell lineages vs just platelets
Progressive marrow fibrosis
Myelofibrosis - low OR high WCC and platelets depending on disease stage

35
Q
A

Malignant haematology (16 decks)

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