Case
A 26-year-old female was diagnosed about a year ago with stage IIA ALK+
ALCL with right axillary and cervical lymphadenopathy and was initiated
on CHOEP (cyclophosphamide, doxorubicin, etoposide, vincristine and
prednisone) chemotherapy. She achieved complete remission following
three cycles of treatment. Soon thereafter, she developed sudden loss of
vision in both eyes associated with fever, and night sweats. Vision loss
started in the left eye then progressed to the right eye, with severity
at a loss of light perception level. She was transferred to our
institution. Significant clinical findings include loss of vision
bilaterally with limited ability to appreciate light and dark stimulus.
Pupils were dilated bilaterally both with a positive direct and indirect
light reflex. She had left ocular dysmotility consistent with CN6 palsy.
No focal weakness or sensory deficit bilaterally in the face and
extremities. She had no lymphadenopathy, or hepatosplenomegaly. Serum
lactate dehydrogenase enzyme level was elevated at 406 U/L (122-222U/L).
MRI brain imaging showed bilateral nodular leptomeningeal enhancements
in the posterior fossa and enhancement around both optic nerves (Fig.1
Before treatment) consistent with neurolymphomatosis. CSF cytology was
positive for atypical large cells consistent with ALCL (Fig.2). Bone
marrow biopsy did not show any involvement with lymphoma. She did not
have any clinical evidence of neurolymphomatosis involving peripheral
nerves. Review of her outside right axillary lymph node biopsy (Fig.3)
confirmed the diagnosis of ALCL with the proliferation of large,
atypical lymphocytes with horseshoe-shaped nuclei on hematoxylin and
eosin stain and strong expression of ALK-1, CD5, and CD30, and negative
expression of PAX 5, CD20, or CD3 by immunohistochemistry studies.
She was initially treated with dexamethasone 40 mg/day for four days
followed by a taper and the Ferrari regimen with high-dose methotrexate
3500mg/m2 and high-dose cytarabine 2000mg/m2 with intrathecal
methotrexate 12.5mg. This was followed with10 sessions of radiation to
the bilateral orbits and optic nerves, a total cumulative dose of
3000cGy. There was no significant improvement in her vision after the
initial treatment. She continued with the Ferrari regimen every 3 weeks
for a total of 4 cycles. After 2 cycles there was no radiological
evidence of residual lymphoma on follow up brain MRI imaging (Fig.1 Post
treatment). Furthermore, repeat CSF cytology was negative for malignant
cells. The patient subsequently underwent high-dose chemotherapy with
BCNU and thiotepa followed by autologous stem cell transplant. One year
after her transplant, she continues to be in complete remission for both
systemic and CNS disease, and had small improvement in light and colour
perception. In spite of her CNS disease being responsive to treatment
and achieving complete remission her vision never improved
significantly.