Lymphoma and myeloma

Question 1

B cell development cycle

Answer 1

• Stem cells become immature B-cells
• Immature B cells mature in the lymphoid tissues
• In the lymph nodes they become centroblasts
• T cells mediate a response which means B cells are released as plasma or memory cells

Question 2

How do myelomas arise

Answer 2

• Arise when maturing plasma cells become stuck in that state and remain immature - located in bone marrrow

Question 3

5 major fractions of electrophoresis

Answer 3

Serum albumin
Alpha-1 globulins
Alpha-2 globulins
Beta globulins 
Gamma globulins
- Serum albumin is the normal major fraction

Question 4

Electrophoresis in malignancy

Answer 4

• Releases one globulin type (paraprotein)
• A spike of one type = M spike
• Other types are reduced

Question 5

Myeloma definition

Answer 5

Incurable malignant disorder caused by malignant proliferation of plasma cells in the bone marrow

Question 6

asymptomatic MGUS

Answer 6

• Monoclonal gammopathy of undetermined significance

- Precedes myeloma

Question 7

Diagnosis of myeloma

Answer 7

• Clonal bone marrow plasma cells >10%

- AND any one or more CRAB features or Myeloma defining events

Question 8

CRAB features

Answer 8

C = hypercalcaemia (>2.75mmol/L)
R = renal insufficiency (creatinine clearance)
A = anaemia (HB<100g/L)
B = bone lesions (one or more osteolytic lesions)

Question 9

MDEs

Answer 9

• > 60% clonal plasma cells on BM biopsy
• Serum-free light chain ratio >100mg/L
• > 1 focal lesion on MRI measuring >5mm

Question 10

Clinical features of myeloma

Answer 10

• Nephropathy - Treatment for myeloma is nephrotoxic
• Increased light chains clog the glomeruli
• Toxic effects on the body

Question 11

Bloods for myeloma

Answer 11

• Rouleux (RBC stack like coins) - indicates high plasma protein number
• Protein electrophoresis to look for paraprotein
• U&E’s looking for renal failure
• Holey skull as plasma proteins inhibit osteoblast activity

Question 12

Management of myeloma

Answer 12

• Steroids - protects kidneys and prevents AKI
• Hydration
• Avoid nephrotoxic drugs

Question 13

amyloidosis

Answer 13

• Rare complication of myeloma
• Light chains are misformed by plasma cells - these aggregate and deposit fibrins in organs.
• Low albumin
• Macroglossia – deposits of amyloid into tongue causing it to become large

Question 14

Waldenstrom’s macroglobulinaemia

Answer 14

• Type of non-Hodgkin lymphoma

- Complication of myeloma with non PCL progression

Question 15

Lymphoma definition

Answer 15

• Malignant proliferation of lymphocytes with predominantly lymph node involvement
• More B cell types than T cell types

Question 16

Hodgkin’s lymphoma =

Answer 16

Presence of Reed-Sternberg cells (owl-eye looking)

Question 17

Non-Hodgkin lymphoma =

Answer 17

• No Reed-Sternberg cells – either aggressive or indolent (slow onset, low turnover, not very curable but asymptomatic anyway)

Question 18

Follicular lymphoma

Answer 18

• Non-Hodgkin
• Increases with age, M=F
• slowly enlarging lymph nodes
• Acquired chromosomal t(14:18) causing overexpression of BCL2
• Diagnosis using FLIPI (follicular international prognostic index)

Question 19

Hodgkin’s lymphoma features

Answer 19

• Itchy (particularly after showering), breathlessness (if nodes around lungs affected)
• Presence of Reed-Sternberg cells (HRS)
• Management = chemotherapy, radiotherapy

Question 20

Acute leukaemia

Answer 20

• Rapid onset
• Dramatic presentation: severe sepsis/Bleeding
• Life-threatening without urgent treatment

Question 21

Chronic leukaemia

Answer 21

• Insidious onset
• Can be incidental finding in otherwise asymptomatic patients
• Don’t always require treatment – active monitoring

Question 22

Myeloid leukaemia

Answer 22

• Cells develop from neutrophils, eosinophils, monocytes, basophils

Question 23

Lymphoid leukaemia

Answer 23

• Cells which develop from lymphocytes; B and T cells

Question 24

Chronic Lymphocytic leukaemia definition

Answer 24

• Malignant disorder of mature B cells – predominantly in the blood and bone marrow

Question 25

Chronic Lymphocytic leukaemia features

Answer 25

• Most common leukaemia in the UK
• Smudge cells characteristic of CL
• Complications = CLL -> advanced lymphoma
• Diagnosis = Binet system-Hb levels, platelet levels
• Presentation - splenomegaly, systemic symptoms, lymphadenopathy