Lymphoid and Myeloid Disorders - Haematological disorders

Question 1

What occurs in acute leukaemia?

Answer 1

Proliferation of primitive precursor cells usually only found in bone marrow
Prolif without differentiation

Replaces normal bone marrow cells, leads to:

• Anaemia: palor and lethargy
• Neutropenia: infecs
• Thrombocytopenia: bleeding
• Bone pain due to marrow infiltration

Question 2

What occurs in acute lymphoblastic leukaemia?

Answer 2

Malignant proliferation of lymphoblasts in bone marrow
Affects mainly children
Good prognosis – 85% cure rate
Esp girls, age 1-10, low WBC, certain morphology
Poor prognosis in adults

Question 3

Treatment of acute lymphoblastic leukaemia?

Answer 3

Induction chemo
Consolidation chemo +/- craniospinal irradiation
Maintenance chemo
Bone marrow transplant if relapse

Question 4

What is acute myeloid leukaemia?

Answer 4

Malignant proliferation of myeloblasts in bone marrow
Affects mostly adults
Poor prognosis 15-50% 5year survival (depends on subtype)
Most patients relapse
Gum infiltration in acute monocytic subtype (M5)

Question 5

Treatment of acute myeloid leukaemia?

Answer 5

Cyclical high dose chemotherapy (induction and consolidation with no maintenance)
Sometimes bone marrow transplantation

Question 6

What is chronic lymphatic leukaemia?

Answer 6

Proliferation of mature lymphocytes, usually B cells
Commonest leukaemia
Affects elderly >65years
Presents with anaemia, infections, lymphadenopathy, splenomegaly
Lymphocytosis on blood film
Good prognosis
Survival >10 years is the norm
Treat only advanced disease

Question 7

Features of chronic myeloid leukaemia?

Answer 7

Gradual onset
High white cell count & splenomegaly
Philadelphia chromosome (t9;22)
BCR-ABL tyrosine kinase
3 phases: Chronic, Accelerated and Blast crisis

Question 8

Treatment of chronic myeloid leukaemia?

Answer 8

Imatinib (Glivec)
Blocks abnormal BCR-ABL tyrosine kinase activity
Can result in molecular remission
Allogeneic stem cell transplantation

Question 9

What is myelodysplasia?

Answer 9

“Premalignant” condition of haemopoietic precursors
Disease of the elderly
Can be asymptomatic
May present with anaemia, thrombocytopenia, pancytopenia
Several subtypes
Variable course
Can transform to acute myeloid leukaemia

Question 10

How to treat myelodysplasia?

Answer 10

Supportive care

Bone marrow transplantation in the young

Question 11

How does hodgkin lymphoma present?

Answer 11

Painless lymphadenopathy

B symptoms: Sweats, Weight loss, Fever

Question 12

Features of hodgkin lymphoma?

Answer 12

Two peaks 15-35yrs and >55
10year survival 90%
4 subtypes
Reed-Sternberg cell diagnostic on biopsy.

Question 13

Treatment of hodgkin lymphoma?

Answer 13

Chemotherapy
Radiotherapy
Stem cell transplantation

Question 14

Features of low grade/indolent non hodgkin lymphoma e.g. follicular lymphoma?

Answer 14

Gradual onset, usually advanced at presentation
May be asymptomatic
Incurable, median survival 10years
Treatment: Chemotherapy, radiotherapy, transplantation

Question 15

Features of aggressive/high grade non hodgkin lymphoma e.g. diffuse large B cell lymphoma?

Answer 15

Rapidly progressive
Usually symptomatic
Potentially curable
Treatment: Chemotherapy, radiotherapy, transplantation

Question 16

List the myeloproliferative disorders

Answer 16

Polycythaemia Rubra Vera (PRV)
- Red cell proliferation

Chronic Myeloid Leukaemia (CML)
- White cell proliferation
Essential

Thrombocythaemia (ET)
- Platelet proliferation

Myelofibrosis
- Marrow stroma proliferation

Question 17

What is polycythaemia/erythrocytosis?

Answer 17

Increase in circulating red cell conc

Question 18

Secondary erythrocytosis?

Answer 18

Hypoxic: High altitude, lung or cyanotic heart disease

Inappropriate erythropoietin secretion: Renal tumour

Question 19

Primary erythrocytosis?

Answer 19

Polycythaemia rubra vera
Pruritus, plethoric facies
Thrombosis
Splenomegaly, hepatomegaly
Treatment: Venesection, aspirin, myelosuppression
Outcome: Myelofibrosis (15-20%), AML (2-10%)

Question 20

Secondary thrombocytosis?

Answer 20

Bleeding, Infection, Inflammation, malignancy

Question 21

Primary trombocytosis?

Answer 21

Uncontrolled malignant proliferation of megakaryocytes
Platelets >600 persistently
Arterial and venous thrombosis
Bleeding with very high counts eg >1500
Treatment with aspirin, hydroxycarbamide, anagrelide, interferon

Question 22

What is multiple myeloma?

Answer 22

Malignant proliferation of plasma cells in the bone marrow

Plasma cells are terminally differentiated B lymphocytes that produce immunoglobulin

Myeloma has monoclonal immunoglobulin in blood and urine

Question 23

Symptoms of myeloma?

Answer 23

Lytic lesions in bones
pain and pathological fractures
Hypercalcaemia due to bone resorption 
thirst,  polyurea, confusion, constipation
Hyperviscocity due to immunoglobulin
Renal failure
Anaemia
Infections

Question 24

How to diagnose myeloma?

Answer 24

Blood and urine tests
X-ray and CT/MRI
Bone marrow

Question 25

How to treat myeloma?

Answer 25

Chemotherapy
Thalidomide, bortezomib
Radiation
Stem cell transplantation