Lymphoma and myeloma Flashcards
B cell development cycle
- 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
How do myelomas arise
- Arise when maturing plasma cells become stuck in that state and remain immature - located in bone marrrow
5 major fractions of electrophoresis
Serum albumin Alpha-1 globulins Alpha-2 globulins Beta globulins Gamma globulins - Serum albumin is the normal major fraction
Electrophoresis in malignancy
- Releases one globulin type (paraprotein)
- A spike of one type = M spike
- Other types are reduced
Myeloma definition
Incurable malignant disorder caused by malignant proliferation of plasma cells in the bone marrow
asymptomatic MGUS
- Monoclonal gammopathy of undetermined significance
- Precedes myeloma
Diagnosis of myeloma
- Clonal bone marrow plasma cells >10%
- AND any one or more CRAB features or Myeloma defining events
CRAB features
C = hypercalcaemia (>2.75mmol/L) R = renal insufficiency (creatinine clearance) A = anaemia (HB<100g/L) B = bone lesions (one or more osteolytic lesions)
MDEs
- > 60% clonal plasma cells on BM biopsy
- Serum-free light chain ratio >100mg/L
- > 1 focal lesion on MRI measuring >5mm
Clinical features of myeloma
- Nephropathy - Treatment for myeloma is nephrotoxic
- Increased light chains clog the glomeruli
- Toxic effects on the body
Bloods for myeloma
- 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
Management of myeloma
- Steroids - protects kidneys and prevents AKI
- Hydration
- Avoid nephrotoxic drugs
amyloidosis
- 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
Waldenstrom’s macroglobulinaemia
- Type of non-Hodgkin lymphoma
- Complication of myeloma with non PCL progression
Lymphoma definition
- Malignant proliferation of lymphocytes with predominantly lymph node involvement
- More B cell types than T cell types
Hodgkin’s lymphoma =
Presence of Reed-Sternberg cells (owl-eye looking)
Non-Hodgkin lymphoma =
- No Reed-Sternberg cells – either aggressive or indolent (slow onset, low turnover, not very curable but asymptomatic anyway)
Follicular lymphoma
- 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)
Hodgkin’s lymphoma features
- Itchy (particularly after showering), breathlessness (if nodes around lungs affected)
- Presence of Reed-Sternberg cells (HRS)
- Management = chemotherapy, radiotherapy
Acute leukaemia
- Rapid onset
- Dramatic presentation: severe sepsis/Bleeding
- Life-threatening without urgent treatment
Chronic leukaemia
- Insidious onset
- Can be incidental finding in otherwise asymptomatic patients
- Don’t always require treatment – active monitoring
Myeloid leukaemia
- Cells develop from neutrophils, eosinophils, monocytes, basophils
Lymphoid leukaemia
- Cells which develop from lymphocytes; B and T cells
Chronic Lymphocytic leukaemia definition
- Malignant disorder of mature B cells – predominantly in the blood and bone marrow
Chronic Lymphocytic leukaemia features
- 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