Lymphoid and myeloid disorders

Question 1

What is acute leukaemia?

Answer 1

• Proliferation of primitive precursor cells usually only found in bone marrow.
• Proliferation without
• Replaces normal bone marrow cells, leads to:
• Anaemia: Palor and lethargy
• Neutropenia: Infections
• Thrombocytopenia: bleeding

Bone pain due to marrow infiltration

Question 2

How do we classify ACUTE Lymphoid and Myeloid Leukaemia’s

Answer 2

Acute & Lymphoid Leukaemia = ACUTE LYMPHOBLASTIC LEUKAEMIA

Acute & Myeloid = ACUTE MYELOID LEUKAEMIA

Question 3

How do we classify CHRONIC Lymphoid and Myeloid Leukaemia’s

Answer 3

Chronic & Lymphoid Leukaemia = CHRONIC LYMPHATIC LEUKAEMIA

Chronic & Myeloid = CHRONIC MYELOID LEUKAEMIA

Question 4

What is acute lymphoblastic leukaemia

Answer 4

• Malignant proliferation of lymphoblasts in bone marrow
• Affects mainly children
• Good prognosis - 85% cure rate (esp girls age 1-10). Poor prognosis in adults

Question 5

How do we treat Acute lymphoblastic leukaemia

Answer 5

• Induction chemotherapy
• Consolidation chemotherapy +/- craniospinal irradiation
• Maintenance chemotherapy
• Bone marrow transplantation only if relapse

Question 6

What is acute myeloid leukaemia

Answer 6

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

Question 7

How do we treat acute myeloid leukaemia?

Answer 7

• Cyclic high dose chemotherapy (induction and consolidation with NO maintenance)
• Sometimes bone marrow transplantation

Question 8

What is Chronic lymphatic leukaemia?

Answer 8

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

Question 9

What is Chronic myeloid leukaemia

Answer 9

• Gradual onset
• High white cell count & splenomegaly
• Philadelphia chromosome (t9;22) (translation between chromosome 9 and 22)
• BCR-ABL tyrosine kinase (2 oncogenes with translocation)
• 3 phases: chronic, accelerated and blast crisis

Question 10

How do we treat chronic myeloid leukaemia?

Answer 10

• Imatinib (Glivec)

• Blocks abnormal BCR-ABL tyrosine kinase activity
• Can result in molecular remission

• Allogeneic stem cell transplantation

Question 11

What is myelodysplasia

Answer 11

• Premalignant condition of haemopoietic precursors
• A disease of the elderly. Can be ASYMPTOMATIC
• May be present with anaemia, thrombocytopenia, pancytopenia
• Several subtypes. Variable course
• Can transform to acute myeloid leukaemia
• Treat with supportive care and bone marrow transplantation in the young.

Question 12

What are the two types of lymphoma?

Answer 12

1. ) Hodgkin lymphoma

2. ) Non-hodgkin lymphoma

Question 13

Describe Hodgkin lymphoma

Answer 13

• Painless lymphadenopathy
• B symptoms (advanced): sweats, weight loss, fever
• 4 subtypes
• Reed-Sternberg cell diagnostic on biopsy
• Treatment:
• Chemotherapy
• Radiotherapy
• Stem cell transplantation

Question 14

Describe Low grade Non Hodgkin lymphoma

Answer 14

• Gradual onset
• May be asymptomatic
• Incurable (10yrs survival)

• Treatment: Chemotherapy, Radiotherapy, Transplantation

Question 15

Describe High grade Non-Hodgkin lymphoma

Answer 15

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

Question 16

What is the disorder involving uncontrolled red cell proliferation

Answer 16

• Polycythaemia Rubra Vera (PRV)

Question 17

What is the disorder involving white cell proliferation

Answer 17

Chronic Myeloid Leukaemia

Question 18

What is the disorder involving platelet proliferation

Answer 18

Essential Thrombocythaemia

Question 19

What is marrow stroma proliferation

Answer 19

Myelofibrosis

Question 20

What is another name for polycythaemia? Describe the two main ways of acquiring it

Answer 20

Erythocytosis

1. ) PRIMARY:
   - Polycythaemia rubra vera
   - Pruritus, plethoric facies
   - Thrombosis
   - Splenomegaly, hepatomegaly
   - Treatment: Venesection, aspirin, myelosuppression

• Outcome: Myelofibrosis (15-20%), AML (2-10%)

2. ) SECONDARY:
   - Hypoxic: High altitude, lung or cyanotic heart disease
   - Inappropriate erythropoietin secretion: renal tumour.

Question 21

What is thrombocytosis

Answer 21

Primary: Essential thrombocythaemia

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

Secondary:
- Bleeding, infection, inflammation, malignancy

Question 22

What is multiple myeloma

Answer 22

• Malignant proliferation of plasma cells (terminally differentiated B cells that produce immunoglobulin) in the bone marrow
• 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, pylori, confusion, constipation

• Hyperviscocity (thick blood) due to immunoglobulin
• Renal failure
• Anaemia
• Infections

Question 24

How do we diagnose and treat myeloma?

Answer 24

DIAGNOSIS:

• Blood and urine tests
• x-rays and CT/MRI
• Bone marrow

TREATMENT:

• Chemotherapy
• Thalidomide
• Radiation
• Stem cell transplantation