Pathology EMQ template

YourName: mark.peterzan@imperial.ac.uk

YourIdentifier: map103

Theme: Plasma cell tumours

OPTION LIST

A	Seligmann’s disease	I	Fanconi syndrome
B	Franklin’s disease	J	Hyperviscosity syndrome
C	POEMS syndrome	K	MGUS
D	Waldenstrom’s macroglobulinaemia with Schnitzler’s syndrome	L	IgM-secreting myeloma
E	High-dose melphalan with autologous stem-cell transplant	M	Serum creatinine
F	Low-dose melphalan with prednisolone	N	Acute monocytic leukaemia
G	Mediterranean lymphoma	O	Light chain secreting myeloma
H	Non-myeloablative chemotherapy with allogeneic stem-cell transplant	P	DiGuglielmo’s disease

For each scenario below, choose the most appropriate answer from the list above. Each option may be used once, more than once or not at all.

1. A 55 year old male of Mediterranean origin presents with chronic diarrhoea, weight loss, and his FBC, U+E shows evidence of malabsorption of iron, folate, calcium, bile acids and cobalamin. Antibiotics to eliminate small bowel infestation do not help. CT shows extensive para-aortic and mesenteric lymphadenopathy. Duodenal biopsy shows lymphoplasmacytoid cell infiltration. Electrophoresis is performed but no monoclonal spike in the gamma region is seen: only the usual smear. Urine light chains are not present. Serum viscosity is 1.8 (normal). Serum immunofixation identifies a clone of truncated alpha heavy chains. He is treated successfully with combination chemotherapy and antibiotics.

2. A 64 year old male presents complaining of recurrent nosebleeds, gum bleeding, fatigue, dizziness, episodes of tingling in the feet and seeing double. On questioning he admits to recurrent cellulitis, red itchy patches of skin and ear infections in the past few months. Fundoscopy shows engorged retinal veins and haemorrhagic spots. He is afebrile but his ESR is raised inappropriately. He is anaemic, has an enlarged spleen and liver, palpable inguinal adenopathy and has reduced sensation and power in the region of spinal roots L4-L5. Skeletal X-rays show no lytic bone lesions. Serum electrophoresis shows IgM paraproteinaemia and urine light chains are found. He enjoys marked symptomatic improvement upon isovolumic plasmapheresis.

3. A 49 year old male presents with E coli pyelonephritis. He has recently noticed increased thirst, polyuria, and recurrent UTIs. After treatment of this episode, his urine is dipstick positive for glucose and negative for protein. He is normoglycaemic upon glucose challenge. A dehydration challenge reveals impaired urinary acidification that does not respond to ddAVP. His anion gap is reduced, serum chloride is high and serum Na low. Abnormalities of lower urinary tract anatomy are excluded. Cationic IgG paraprotein is confirmed by serum electrophoresis and immunofixation. Marrow tap reveals clonally proliferating cells that are CD38+, CD138+, CD19-, CD20- and have immunoglobulin heavy chain translocations detectable by FISH.

4. The patient in question 3 receives supportive therapy (hydration, bisphosphonates, vitamin D, EPO, vaccinations) and therapy targeting his tumour cells. Given his young age, which regimen should he receive?

5. The patient in question 3 is inappropriately treated by a doctor who is not up-to-date. He is put on chronic alkylating agent therapy. It seems to control the paraprotein but five years later he returns with Strep pneumoniae pneumonia, anaemia, thrombocytopaenia, and a white cell count of 100. Marrow tap reveals erythroblastic clonal change.

ANSWERS

1. A

2. D

3. I

4. E

5. P