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Haem > Bone Marrow Disorders > Flashcards

Bone Marrow Disorders Flashcards

1
Q

Pancytopenia causes

A

Aplastic anaemia
Infiltration
Megaloblastic anaemia
Myelofibrosis

Hypersplenism

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2
Q

What is agranulocytosis

A

Granulocytes not made possibly due to drugs (carbimazole, procainamide, sulphonamides, clozapine, dapsone)
Pt vulnerable to neutropenia and infection

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3
Q

Bone marrow biopsy types

A

Aspirate provides a film

Trephine provides core which can assess cellularity, architecture + infiltration

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4
Q

What is aplastic anaemia

A

Rare stem cell disorder where bone marrow stops making cells

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5
Q

Aplastic anaemia causes

A 
Autoimmune
Drug induced
Hep/parvovirus
Irradiation
Inherited in Fanconi anaemia
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6
Q

Aplastic anaemia diagnosis

A

Bone marrow biopsy

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7
Q

Aplastic anaemia treatment

A

Transfuse, neutropenic regimen if needed
Allogeneic marrow transplant if young
Immunosuppression with ciclosporin +antithymocyte globulin may help in autoimmune

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8
Q

Myeloproliferative disorder classification

A 
Classed by which myeloid stem cell is proliferating
RBC -> Polycythaemia vera
WBC -> CML
Platelets -> Essential thrombocythaemia
Fibroblasts -> Myelofibrosis
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9
Q

What is polycythaemia vera

A

Malignant proliferation of clone derived from a pluripotent stem cell
JAK2 mutation in 95%

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10
Q

Polycythaemia vera presentation

A 
>60yrs typically
Hyperviscosity
Itching after hot bath
Burning in fingers + toes
Facial plethora
Splenomegaly
Gout from urate from RBC turnover
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11
Q

Polycythaemia vera investigations

A

Marrow shows hypercellularity with erythroid hyperplasia
Cytogenetics to differentiate from CML
Dec serum EPO
Raised RBC mass on Cr51 studies in normal PaO2

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12
Q

Polycythaemia vera treatment

A

Aim to keep HCT<0.45 to prevent thromboses
Venesection in young
Hydroxycarbamide in older
alpha-interferon in women on childbearing age
Aspirin 75mg daily

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13
Q

Essential thrombocythaemia treatment

A

Aspirin 75mg OD

Hydroxycarbamide in high-risk pts

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14
Q

What is myelofibrosis

A

Hyperplasia of megakaryocytes which produce platelet derived growth factor, causing marrow fibrosis + extramedullary haematopoiesis

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15
Q

Myelofibrosis presentation

A

Hypermetabolic symptoms: night sweats, fever, weight loss
Abdo discomfort from splenomegaly
Bone marrow failure

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16
Q

Myelofibrosis tests

A

Leukoerythroblastic cells (nucleated RBCs) on blood film
Teardrop RBCs
Hb decreased
Bone marrow trephine for diagnosis

17
Q

Myelofibrosis treatment

A

Marrow support

Allogeneic stem cell transplant in young may be curative

18
Q

Myelofibrosis prognosis

A

Median survival 4-5 yrs

Haem (10 decks)

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