Case 135 – Update 2

Our patient was discharged home and took 60mg Prednisolone for 3 weeks.

She successfully started a steroid wean following this and is slowly reducing down to 30mg of prednisolone daily at present.

Her Hb has normalised at 130g/l with a normal LDH 205. Her platelet count is 235, neu 3, lymph 5. Her renal and liver function are all within normal ranges. She had a small IgM kappa PP 2g/l detected and is negative for hepatitis and HIV. Her CT scan showed 19cm splenomegaly and no lymphadenopathy.

She had no B symptoms and reported no discomfort from the large spleen.

Her bone marrow biopsy showed she had a clonal population of B lymphocytes that were CD19 + CD5+/- CD 10 – CD103 – CD79b + sIg ++

MYD88 negative CCND1 negative

Bone marrow trephine showed infiltrate with intrasinusoidal pattern.

The MDT agreed the likely diagnosis was Splenic marginal zone lymphoma with associated AIHA.

Does she require any treatment for her SMZL at present?

If no treatment planned what features would make you treat?

She is asking you about prognosis – What prognostic scores can you use in SMZL?

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