Dec 2, 2009
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An interesting case I recently had on my medicine sub-I.

The patient is a 55 year old woman with no significant past medical history who presented with several month history of episodic R visual loss. Several weeks prior to admission the patient saw a neuro-opthalmologist who diagnosed R homonymous hemianopsia. CT scan demonstrated non-specific white matter changes in the R parietal lobe. 2 days prior to admission she saw her PCP who drew labs that were significant for lyme IgM+ (IgG-) and WBC count of 40k (80% lymphs). She was instructed to come to our hospital.

She got an LP that was significant for WBC count of 31 with normal opening pressure.

Initially we thought this was CNS lyme given the IgM+. However, lyme EIA and CSF PCR eventually came back negative and her peripheral smear was grossly abnormal.

Peripheral smear and Flow:
Lymphocytes accounted for the majority of the cellularity (>80%), and are mostly small mature lymphocytes with condensed chromatin, inconspicuous nucleoli and scant amounts of pale blue cytoplasm. Occasional (10-20%) larger forms with open chromatin, prominent nucleoli and more abundant cytoplasm, as well as rare large granular lymphocytes were also identified. Smudge cells are present but inconspicuous.

The lymphocyte region contains surface kappa-restricted CD5- CD10- CD13+ CD19(variable)+ CD20+ CD23- CD25- CD38(variable)+ CD103- IgM+ B cells (79.1% of the region, 58.2% of total events). Both the SmIg and CD20 are bright. Very minor (~2%) of circulating cells are CD10 and/or CD34 positive. Also present are T cells without significant loss of pan-T antigens, with a CD4:CD8 ratio of 2.3:1 (6.1% of the region, 4.5% of total events), as well as CD3- CD5- CD7+ NK cells (1.5% of the region, 1.1% of total events).

In summary, flow cytometry demonstrates an expansion (59% of total events) of surface CD5- CD10- CD13+ CD19(variable)+ CD20+ CD23- CD38(variable)+ IgM+ B cells. The immunophenotype is not specific. Due to the absence of CD5 and CD10 expression, it is unlikely to represent SLL/CLL, mantle cell lymphoma or follicular lymphoma. In addition, the absence of CD25 and CD103 expression make it unlikely to be hairy cell leukemia. The differential diagnosis includes marginal zone lymphoma and lymphoplasmacytic lymphoma; however, the morphology is not prototypic of either splenic marginal zone lymphoma or lymphoplasmacytic lymphoma. In addition the significance of aberrant CD13 expression is unknown (no other myelomonocytic antigens--CD11b, CD14, CD15, CD33, MPO--are expressed). Coexpression of myeloid antigens (CD13 > CD33) has been described in CLL/SLL, with some data indicating an association with poor prognosis.

CSF was also sent for flow with the same circulating non-classifiable small B cells.

We got an MRI of the orbits that showed diffuse bilateral leptomeningeal enhancement surrounding the optic nerves without evidence of atrophy or intrinsic nerve T2 prolongation and signal abnormality in the right posterior centrum semiovale and periventricular region, also seen on prior CT.

And a CT of abd/pelvis: severe splenomegaly, with the spleen measuring 25 cm and pathologically enlarged retroperitoneal lymph nodes.

So, in the end, the outside lyme test was probably a false positive and she has a circulating mature B cell leukemia/lymphoma not otherwise classifiable (BM bx results are pending).

The question is, how do we treat her? The consulting heme/onc team pushed for transfer to our cancer floor for intrathecal chemo. Neuro, who was also following the patient, was very adamant about waiting for the final diagnosis. They claimed that while intrathecal chemo was indicated for lymphoma, if the dx was leukemia, XRT to the optic nerves would indicated to treat her visual symptoms (no chemo).

I don't know yet what she will get because after a few days on the onc service she was discharged with outpatient heme/onc follow up.

I did some hunting around pub med/other resources but couldn't find much on this topic. Found lots of studies on XRT for CNS leukemia (like ppx in kids with ALL) but very little on treating the optic nerves for primary involvement.

Any thoughts?
 

Brim

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An interesting case I recently had on my medicine sub-I.
This lady needs URGENT XRT to the orbits +/- skull base. I'm hoping the heme/onc folks don't dilly-dally around and get her in to see a Rad Onc soon.

She needs this first. Intrathecal chemo can wait.
 

Palex80

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Dec 17, 2007
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One major question that needs to be answered is if any other tests are to be done on this patient.
If you are done with collecting samples to find out what the correct diagnosis is and you are simply waiting for the molecular genetic results, then I would suggest to simply give the patient steroids for starters.
You can't do much wrong with steroids, if you don't want to collect any other samples and it will be a good tolerable and usually highly (temporarily) effective therapy both for a lymphoma and a not so aggressive form of leukemia.
I would expect the steroids to have a good effect on the optic nerves, while you figure out what to do next.


On a side note:
I treated a patient a couple of months ago who was diagnosed with an extranodal marginal zone cell lymphoma of the orbita with involvement of extracranial part of the optic nerve.
The major involvement was on the right side, but the MRIs also showed suspicious enhancement along the left optic nerve too.
We ended up giving him radiation therapy to both orbitae, while trying to keep the dose on the left side a bit lower in order to minimize the risk of a dry eye there. The tumour on the right side was too big, so we could not spare his right lacrimal gland.
Schedule was 18 x 2 Gy, he is currently in complete remission.