Our first case looked at a blood film of a 48 year old gentleman who had presented with neutropenia and monocytopenia. This is a common presentation for patients with hairy cell leukaemia and this leaves them susceptible to opportunistic and bacterial infections.
The blood film allowed us to identify hairy cells – which are typically large (twice the size of normal lymphocytes), have abundant cytoplasm (low nucleus to cytoplasmic ratio), with villous projections. The nucleus is round, oval, or slightly indented, and occasional bilobed. Nuclear chromatin is smooth, with absence of a nucleolus. HCL variant, have similar cytoplasmic features but have a round nucleus with condensed chromatin and distinct nucleolus.
The correct answer for our MCQ was D.
a) Cd20+, CD5 -, CD10-, CD79a +,CD11c+, CD103-, CD123- (splenic marginal zone)
b) CD20 +,CD5+, CD23+, FMC7 -,SmIg weak, CD79b – – (CLL)
c) CD20+, CD5+, CD23-, FMC7+. SmIg weak, cd79b + -(Mantle cell)
d) CD20+ CD5-, CD23-, CD103 +, CD11C +, CD10-, CD123+ (hairy cell)
e) CD20+, CD5-, CD10+, CD19+, CD22+, CD79a+ (follicular)
This case looked at the treatment options for a young women with hodgkins lymphoma. Answering this questions relied upon accurately classifying this patient into early favourable disease.
Early favourable (EORTC)
- No large mediastinal lymphadenopthy
- ESR<50 without B symptoms
- ESR<30 with B symptoms
- Age <50
- 1-3 lymph node sites involved
- large mediastinal lymphadenopathy
- ESR>50 without B symptoms
- ESR>30 with B symptoms
- Age >50
- >4 lymph node sites.
Rationale for treatment:
HD 10 trial – 4 treatment arms
- 2 cycles ABVD + 20 Gy
- 2 cycle ABVD +30 Gy
- 4 cycles ABVD +20Gy
- 4 cycles ABVD +30 Gy
No difference in PFS, or OS was found between these 4 groups, therefore the least toxic approach of 2 x ABVD +20Gy appears to be sufficient in treating early stage disease.
Obviously radiotherapy treatment is not without side effects and therefore some patients may opt not to have radiotherapy (especially young/female with mediastinal disease – increased risk of breast cancer)
In the RAPID trial patients underwent 3 cycles of ABVD, and if PET negative following chemotherapy, did not receive radiotherapy. Patients who remained PET positive recieved a further cycle of ABVD and radiotherapy. The study found that patients had a very good prognosis either with or without radiotherapy.
Therefore in this case either 2xABVD +20 Gy, or 3 cycles of ABVD would be appropriate treatment options.
Our case brought up the question of pregnancy and hodgkins disease – perhaps we’ll explore this a little more in another case!
This blood film demonstrated the characteristic atypical lymphocytes seen in infectious mononucleosis, a disease resulting from primary infection of EBV. The patients had presented with splenic rupture, a rare complication.
This case looked at the clasification of myeloma. The correct answer to the MCQ was active myeloma. Now lets take a look at the criteria.
Clonal bone marrow plasma cells of >10%, or biopsy proven bony or extramedullary plasmacytoma + and one or more of the following CRAB features and myeloma defining events:
- hypercalcaemia (>2.75mmol/L)
- renal insufficiency (crcl <40mL per minute)
- anaemia (Hb <100g/L)
- bone lesions ( one or more osteolytic lesion on radiograph/CT/ PET
Myeloma defining events:
- 60% or greater plasma cell clone
- serum invovled/uninvolved light chain ratio >100
- more than one focal lesion on MRI that is at least 5mm or greater in size.
Postgraduate haematology, sixth edition Hoffbrand,A.V.
Andre, M. P., O. Reman, et al. (2012). “Interim Analysis of the Randomized Eortc/Lysa/Fil Intergroup H10 Trial On Early PET-Scan Driven Treatment Adaptation in Stage I/II Hodgkin Lymphoma.” ASH Annual Meeting Abstracts 120(21): 549-.
Engert, A., A. Plutschow, et al. (2010). “Reduced treatment intensity in patients with early-stage Hodgkin’s lymphoma.” N Engl J Med 363(7): 640-652.
International myeloma working group. http://imwg.myeloma.org/international-myeloma-working-group-imwg-criteria-for-the-diagnosis-of-multiple-myeloma/
Well done to everyone who joined in our mini MCQs! Join us next week for the start of a new case.