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3rd Year Random Notes > Myeloproliferative Disorders and Paraproteinaemias > Flashcards

Myeloproliferative Disorders and Paraproteinaemias Flashcards

1
Q

Name the 3 myeloproliferative disorders

A 
Polycythaemia rubra vera (PRV)
Essential thrombocythaemia (ET)
Idiopathic myelofibrosis (IMF)
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2
Q

What are myeloproliferative disorders?

A

Incurable conditions characterised by a prothrombotic state, bone marrow fibrosis and later development of acute leukaemia

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3
Q

When do myeloproliferative disorders tend to present?

A

> 50 years old

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4
Q

Cause of myeloproliferative disorders?

A

Haemapoietic stem cells acquire a mutation which results in a hypercellular bone marrow and high peripheral blood counts.

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5
Q

Clinical features of myeloproliferative disorders?

A

Often discovered incidentally on FBC
Increased Risk of Arterial and venous thrombosis (ischaemic stroke and DVT/PE/Thrombosis at other sites)
Gout may be a complication

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6
Q

Symptoms and Signs of PRV?

A 
Symptoms:
Headaches
Dizziness
Tinnitus
Visual Disturbance
Itching after hot bath and erythromelalgia, a burning sensation in fingers and toes, are characteristic

Signs:
Facial plethora
Splenomegaly

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7
Q

Investigations for myeloproliferative disorders?

A 
PRV:
FBC (Increased; RCC, Hb, HCT, PCV, often also WBC and platelets)
B12 count
Serum erythropoietin
Raised red cell mass on 51Cr studies
USS to show splenomegaly
Blood Film (Thrombocytosis)
Clonal Markers (JAK-2 mutation 90%)
ET:
Blood Film (Thrombocytosis)
FBC (Platelets >600x10^9cells/l)
Bone marrow examination
Ferritin assay excludes iron deficiency
Clonal Markers (JAK-2 mutation 50%)
IMF:
FBC (Anaemia with pancytopenia)
Bloodfilm (tear drop RBCs)
Bone marrow trephine
Clonal Markers (JAK-2 mutation 50%)
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8
Q

Treatment of PRV?

A

Venesecion to maintain hematocrit (HCT) at <0.45

Aspirin +/- Hydroxycarbamide to maintain platelet counts

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9
Q

Symptoms and signs of ET?

A

Persistently increased platelets (>1000x10^9L)
Bleeding or arterial and venous thrombosis
Headache
Chest Pain
Light Headedness
Erythromelalgia

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10
Q

Treatment of ET?

A

Low risk groups oral aspirin only

Hydroxycarbamide in high risk patients

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11
Q

Symptoms and Signs of IMF?

A 
Night sweats
Fever
Weight Loss
Abdominal Discomfort due to splenomegaly
Bone marrow failure
Characteristic teardrop RBC's found on blood film
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12
Q

Treatment of IMF?

A

Transfusions and splenectomy if very symptomatic

Allogenic bone transplant considered in young patients (high risk)

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13
Q

Prognosis of each disease?

A

Stable low risk PRV or ET: 15-20 years

IMF: 5-7 years

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14
Q

What are paraproteins?

A

Non-functional antibodies, either intact or lightchain only, that originate from a clone of plasma cells or B lymphocytes
(A monoclonal immunoglobulin present in blood or urine)

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15
Q

Common causes of paraproteinaemia?

A

Monoclonal gammopathy of undetermined significance (MGUS)
Myeloma
Solitary plasmocytoma
Lymphoproliferative diseases
Autoimmune disorders are less common causes

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16
Q

Which type of paraproteins indicate which disease?

A

IgM Paraproteins = Lymphoma (Lymphocytes)

IgG and IgA paraproteins = Myeloma (Plasma cells)

17
Q

What is myeloma?

A

A neoplastic disorder of plasma cells, usually resulting in the excessive production of a single type of paraprotein in 98% of cases

18
Q

Clinical features of Myeloma

A 
Bone disease - Osteoclastic Activity:
Lytic bone lesions
Pathological Fractures
Cord Compression
Hypercalcaemia

Bone marrow failure esp. anaemia

Infections

Renal Failure - Cast Nephropathy

Hyperviscosity:
Syndrome caused by increased viscosity in blood, impaired microcirculation and hypoperfusion
Commonest feature is bleeding (retinal, oral, nasal, cutaneous)

Hypogammaglobulinaemia

Amyloidosis

19
Q

Investigations for Myeloma?

A 
FBC
ESR
U's and E's
Creatinine
Urinalysis (Bence-Jones Protein)
Serum paraproteins
MRI and CT in suspected cord compression
Bone marrow aspirate (Shows >10% plasma cells)
staging based on albumin and beta-2 microglobulin
20
Q

Treatment of myeloma?

A

Chemotherapy
Bisphosphonate therapy - Zoledronic acid
Radiotherapy
Steroids
Surgery - Pinning of long bones; decompression of spinal cord
Allogenic stem cell transplant is curative in younger patients

3rd Year Random Notes (4 decks)

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