Case 97 – update 3

Thanks for your help so far with this case.

so far we know our patient is a 54 year old male who presented to clinic with distal sensory loss and a broad based gait which has been slowly progressing over the past 9 months.

He has loss of proprioception, temperature and fine touch symmetrically to around ankle level. He also has got bilateral reduced ankle jerks with preserved knee reflexes.

All the test results are back for our patient and are summarised below:

FBC: Hb 14g/dl others all within normal range

U&E and LFT: Normal

Albumen : 42g/l (normal)

B12 and folate: Normal

Serum electrophoresis: Ig M Kappa paraprotein of 6g/dl.

Serum B2 Microglobulin: 3.2mg/l (Normal <2mg/l).

LDH: 540u/l (reference range 240-450u/l)

24 hour urine protein with electrophoresis : negative.

CT CAP: Nolymphadenopathy or organomegaly.

Bone marrow aspirate report: Plasmacytoid and lymphoplasmacytoid cells are relatively prominent.

Abnormal population: Kappa restricted B cell population = 18% of total nucleated cells.

Immunophenotype: CD19+, CD5 negative, CD23 negative/wk, CD10 negative, CD103 negative, CD11c+/-, CD38+/-, FMC7+, CD79b+, sIg++ (CLL score 0/5)

Molecular studies: The c.794T>C p.(Leu265Pro) mutation in exon 5 of the MYD88 gene is present at low levels.

Bone Marrow trephine: Diffuse infiltrate of small lymphoid cells with reactive mast cell infiltrate noted at peripheries.

(The immunophenotype confirms it is a B cell NHL, however no other markers to assist in identification – No CD 5 for CLL or MCL, no CD10 for FL and no CD103 for HCL despite the occasional lymphocyte with some projections on aspirate. Molecular testing has confirmed the presence of MYD88 mutation seen in up to 90% of LPL cases.)

The neurology team have found the patient is positive for anti MAG Ab

Nerve conduction testing: prolonged conduction velocities consistent with demyelination in an axonal loss pattern.

You plan to present the patient at MDT this week as an LPL with disabling neuropathy secondary to anti MAG Ab

Would you treat this patient?

What are the management options?

The patient wants to know how likely any treatment is to work?

He also asks how fast any treatment will work what would you say?

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