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Hindawi Case Reports in Oncological Medicine Volume 2020, Article ID 3752409, 4 pages https://doi.org/10.1155/2020/3752409 Case Report Stage IV Small Lymphocytic Lymphoma Presenting with Unilateral Vision Loss Christopher Le , Adam Jacob , Devarajan Iyengar, John Dedousis , and Antonios Tsompanidis th Department of Graduate Medical Education, CarePoint Health Bayonne Medical Center, 29 E. 29 Street, Bayonne, NJ 07002, USA Correspondence should be addressed to Christopher Le; email@example.com Received 13 June 2019; Revised 16 December 2019; Accepted 28 December 2019; Published 11 January 2020 Academic Editor: Jeanine M. Buchanich Copyright © 2020 Christopher Le et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. Small lymphocytic lymphoma (SLL) is a manifestation of chronic lymphocytic leukemia (CLL) in which malignant B-cell lymphocytes accumulate in the lymph nodes or bone marrow. In this report, we describe the medical course of a patient diagnosed with stage IV small cell lymphocytic lymphoma, who presented to the emergency room with acute neurologic manifestations of SLL. 1. Introduction 2. Case Presentation SLL is a form of non-Hodgkin lymphoma that involves The patient is a 71-year-old Hispanic male with a past med- malignant proliferation of B-cells in lymph nodes and other ical history of hypertension, COPD, and prostate cancer in lymphoid tissues. SLL is often categorized along with CLL remission post-radiation presented to the emergency depart- because they share many of the same clinical features but ment in May 2017 with a one-month history of neck swelling, diﬀer in the site of B-cell proliferation. CLL/SLL is the most sore throat, and ear pain. CT scan of the neck soft tissue common form of leukemia in the United States, making up showed multiple enlarged cervical lymph nodes in the about 25-30% of all diagnosed leukemia cases . These leu- submandibular, submental, posterior cervical, and jugulodi- kemia cases have a stronger disposition for males compared gastric regions extending into the supraclavicular area to female, with the ratio of cases about 1.5 : 1 , most often (Figure 1). On the exam, the patient had left posterolateral diagnosed in the elderly population > 60 years old. The inci- oral pharyngeal soft tissue enlargement and possible left pal- dence of SLL is very low in patient categorized with CLL, as atine tonsil involvement. Unfortunately, he left against med- about 10% of patient have only nodal involvement. ical advice before any further evaluation could be performed. Most patients present asymptomatically, with leukocyto- In November 2017, the patient returned to the emergency sis found incidentally on routine blood analysis. The patient department with a 2-day history of headache, neck pain, may show symptoms of anemia, thrombocytopenia, chronic painless vision loss in the right eye, and enlarging cervical infections, hepatosplenomegaly, or lymphadenopathy at and inguinal lymphadenopathy. Signiﬁcant ﬁndings on presentation, but these are much less common than the examinations were complete right eye blindness and bilateral asymptomatic patient. More rarely, patients present with nontender lymphadenopathy in the cervical, supraclavicular, classic B-symptoms including fever, unexplained weight loss, and axillary regions. On fundoscopic examination of the or night sweats. Because SLL is an uncommon, indolent right eye, no retinal hemorrhages were appreciated. How- disease, patients are often asymptomatic for years and may ever, there was concern for optic nerve swelling and possible eventually present due to complications of metastases. increased intraocular pressure. 2 Case Reports in Oncological Medicine the blood brain barrier to decrease the size of the intracranial lesion. Thus, palliative local radiation was initiated. Through radiation treatments directed at the lesion on his optic nerve, the patient slowly regained vision in his right eye. 3. Discussion Chronic lymphocytic leukemia (CLL) is caused by the dysregulated growth and diﬀerentiation of malignant white blood cells in the bone marrow. These malignant white blood cells present in the blood often inﬁltrate other organs, such as the liver, spleen, and skin. Small lymphocytic leukemia (SLL) is a variant of CLL, in which most of the malignant white blood cells are present in lymph nodes. A previous study done in 2016 showed that any signiﬁcant central nervous Figure 1: CT of the soft tissue in the neck showing diﬀuse cervical system involvement of CLL/SLL was an extremely rare lymphadenopathy. consequence, with about 80% of the CNS symptoms occur- ring due to other causes . CNS involvement in CLL/SLL Lab work was signiﬁcant for leukocytosis of 47,600/μL is often undiagnosed or does not show any clinical signs or with 86% lymphocytes (10% atypical lymphocytes), symptoms, but patients with CNS involvement have a hemoglobin of 8.3 g/dL, platelet count of 46,000/μL, and median survival time of 12 months after diagnosis . LDH of 4067 U/L. Due to the high leukocytosis and acute Studies have shown that CNS involvement in the progression vision loss, there was concern for a retinopathy secondary of CLL/SLL occur in untreated patients while correlation to hyperviscosity syndrome. However, serum viscosity was with stage, duration, gender, age, WBC count, or immuno- 1.1 cP (range: 1.1-1.8 cP). Furthermore, CTA of the head logic phenotype has not been shown . and neck and carotid ultrasound were unremarkable While the mechanism of spread into the CNS is not well showing no signiﬁcant stenoses, which ruled out hypervis- understood to this point, several cases have been presented cosity syndrome and any vascular etiology for his vision loss. that postulate possible sites of entry into the CNS. The dura MRI of the brain showed no evidence of acute infarction or can act as a barrier to prevent direct invasion into the deeper any enhancing mass lesion, which ruled out any acute layers of the CNS. However, spread into the CNS may come etiology within the brain as the cause of his vision loss. CT as a result of hematogenous or lymphomatous spread that of the chest, abdominal, and pelvis showed extensive medias- perforates dural vessels and nerves into the arachnoid space tinal and retroperitoneal lymphadenopathy with splenomeg- . Some studies have linked integrin CD49d, the α chain aly (Figure 2). MRI of the brain and orbits showed a minimal of the α4β1, with migration of malignant cells across vascular focal area of enhancement within the right optic nerve inside endothelium, showing clinical relevance to disease progres- of the optic canal (Figure 3). sion. In addition, extensive bone marrow inﬁltration has Peripheral blood smear showed normocytic, hypochro- been linked to high CD49d expression . mic red blood cells, lymphocytosis comprised of small and The diﬀerential diagnosis for this patient, who presented large lymphocytes with few atypical lymphocytes, and with sudden onset right eye vision loss, is broad. Important thrombocytopenia without clumping. Biopsy was obtained diagnoses to consider include optic neuropathy, optic neuri- from a paraaortic lymph node instead of the enlarged cervical tis, autoimmune disorders, inﬂammatory pseudotumors, lymph nodes to diﬀerentiate prostate cancer metastasis from and other compressive neuropathies, including optic nerve a primary malignancy such as leukemia or lymphoma, as glioma, optic nerve meningioma, optic nerve or orbital prostate cancer is unlikely to metastasize in cervical lymph metastases, inherited disorder, and toxic, nutritional, or nodes. The biopsy was consistent with B-cell chronic metabolic optic neuropathies . Urgent ophthalmology lymphocytic leukemia. Flow cytometry was positive for consult may be necessary in order to inﬂuence the diﬀeren- CD38 and ZAP-70. Bone marrow biopsy showed chronic tial, ordering of subsequent testing, and prevention of lymphocytic leukemia greater than 90% of the cellularity with further progression of the disease. If there is high clinical near total displacement by small lymphocytic inﬁltrate. suspicion of CLL/SLL involvement in the optic nerve, imme- Fluorescence in situ hybridization was positive for trisomy diate orbital radiation and induction chemotherapy may be 12 and deletion of TP53 on chromosome 17 at p13. Immuno- necessary to preserve vision. MRI of the brain and orbit histochemical analysis of a paraaortic lymph node was should be done as soon as possible to narrow down the positive for CD20, PAX5, CD5, CD23, BCL2, and CD43, diﬀerential, visualize any possible compressive lesions, and determine location of the disease . but negative for Cyclin D1 and p53. The patient was diagnosed with extensive stage IV small Optic nerve biopsy can also be considered; however, this cell lymphocytic lymphoma with inﬁltration of the right carries the risk of permanent vision loss and results may vary posterior optic nerve. The patient was started on a regimen due to small biopsy collection in order to preserve vision . of rituximab and bendamustine. However, there was a In a study performed by Levin et al., 14 of 15 patients that underwent optic nerve biopsy were able to get a deﬁnitive concern that chemotherapy would not adequately penetrate Case Reports in Oncological Medicine 3 26 .09 mm 24 .68 mm (a) (b) 103 .26 mm 147 .74 mm 30 .03 mm (c) (d) Figure 2: CT of the chest, abdomen, and pelvis. (a) Axillary lymphadenopathy. (b) Retroperitoneal lymphadenopathy. (c) Paraaortic lymphadenopathy. (d) Splenomegaly. showed the ﬁbrovascular septa of the optic nerve inﬁltrated by lymphocytes and histiocytes . Optic nerve biopsy results were also presented in a case by Myers et al. in a patient with recurrent vision loss and a history of T lymphoblastic lymphoma in remission. The biopsy showed lymphoblastic inﬁltration with atypical lymphoid cells with morphology like the original disease . Standard treatment for early, stable CLL disease is obser- vation monitoring. For initial management of CLL in advanced and active disease, ibrutinib (NCCN Category 1) or venetoclax plus obinutuzumab (NCCN Category 2A) are preferred treatment options regardless of del(17p)/TP53 mutation status. Other alternative ﬁrst-line therapy options include chlorambucil plus anti-CD20 antibody (NCCN Category 2A; ESMO Grade A, Level 1) or bendamustine plus anti-CD20 monoclonal antibody (ESMO Grade B, Level 1). Figure 3: MRI of the brain and orbits with contrast showing Our patient was placed on rituximab and bendamustine as lymphoma inﬁltration in the right optic nerve. it was thought to be better tolerated in an older patient than ibrutinib due to its toxicities with his multiple comorbidities diagnosis. Of those patients, 4 who had an unaﬀected fellow and poor prognosis. For patients with SLL, regional radiation eye were prevented from further vision loss, and 6 patients is often used. Lymphomas are among the group of tumors with tumors had the etiology of their vision loss conﬁrmed that are exquisitely sensitive to radiation therapy. In a study . Kim et al. presented two cases in which patients with done by Johannsson et al., patients with disseminated CLL and indolent non-Hodgkin lymphoma (iNHL) were treated B-cell non-Hodgkin lymphoma had optic nerve involvement and underwent optic nerve biopsy. Results of one biopsy with low-dose radiotherapy (4 Gy in 2 fractions) for palliative showed mononuclear cells invading the ﬁbrovascular pial treatment. Patients with CLL had an overall response rate of septa surrounding bundles of necrotic ﬁbers. The other 71% (29% complete response, 42% partial response) with a 4 Case Reports in Oncological Medicine median duration of response of about 22 months and no ture review,” Journal of Neuro-Oncology, vol. 106, no. 1, pp. 185–200, 2012. signiﬁcant side eﬀects. The study concluded that low-dose radiotherapy is a highly eﬀective palliative treatment for  K. Rakul Nambiar, T. Anoop, and N. Prakash, “CLL with CNS involvement-a diagnostic dilemma,” Acta Scientiﬁc Cancer masses in patients with disseminated CLL and iNHL . Biology, vol. 1, no. 1, pp. 17–19, 2017. In a study done by Pacelli et al., rare orbital tumors treated with radiotherapy had subjective and objective improvement  J. L. Kim, P. R. Mendoza, A. Rashid, B. Hayek, and H. E. Grossniklaus, “Optic nerve lymphoma: report of two cases of visual and ocular symptoms without evidence of disease and review of the literature,” Survey of Ophthalmology, progression . vol. 60, no. 2, pp. 153–165, 2015. Our case showed the rapid progression of the disease  C. M. Rojas-Hernandez, J. Nemunaitis, K. D. Marjon, within the span of 6 months from the ﬁrst clinically reported D. Bustamante, Q.-Y. Zhang, and J. M. Gillette, “Chronic symptoms. The patient experienced severe neurologic symp- lymphocytic leukemia with clinical debut as neurological toms, including complete right-sided, painless vision loss and involvement: a rare phenomenon and the need for better right-sided headache and neck pain. The patient had a his- predictive markers,” BMC Hematology, vol. 17, no. 1, 2017. tory of lymphadenopathy without a diagnosis of CLL/SLL,  G. Ahle, V. Touitou, N. Cassoux et al., “Optic nerve inﬁltration further complicating the case. While the clinical manifesta- in primary central nervous system lymphoma,” JAMA Neurol- tions of this type of leukemia can be variable, CNS involve- ogy, vol. 74, no. 11, pp. 1368–1373, 2017. ment provides a particularly unfavorable prognosis.  K. A. Myers, A. Nikolic, K. Romanchuk et al., “Optic neu- However, the role of CNS involvement in SLL should be ropathy in the context of leukemia or lymphoma: diagnostic studied further to better understand disease progression. approach to a neuro-oncologic emergency,” Neuro-Oncology Practice, vol. 4, no. 1, pp. 60–66, 2017. 4. Conclusion  M. Dayan, J. Elston, and B. McDonald, “Bilateral lymphoma- tous optic neuropathy diagnosed on optic nerve biopsy,” SLL is an indolent form of lymphoma and patients often Archives of Ophthalmology, vol. 118, no. 10, pp. 1446–1449, experience asymptomatic lymphadenopathy with no other symptoms. While cytopenias and bone marrow failure are  M. H. Levin, J. J. Ney, S. Venneti et al., “Optic nerve biopsy in common, neurologic manifestations of SLL are very rare the management of progressive optic neuropathy,” Journal of but should be included in the diﬀerential of patients with Neuro-Ophthalmology, vol. 32, no. 4, pp. 313–320, 2012. sudden onset neurological symptoms and progressive  J. Jóhannsson, L. Specht, J. Mejer, and B. A. Jensen, “Phase lymphadenopathy. II study of palliative low-dose local radiotherapy in dissemi- nated indolent non-Hodgkin's lymphoma and chronic lymphocytic leukemia,” International Journal of Radiation Consent Oncology � Biology � Physics, vol. 54, no. 5, pp. 1466–1470, Informed consent was obtained from the patient’s sister as he 2002. is now deceased.  R. Pacelli, L. Cella, M. Conson et al., “Fractionated stereo- tactic radiation therapy for orbital optic nerve sheath meningioma–a single institution experience and a short Conflicts of Interest review of the literature,” Journal of Radiation Research, vol. 52, no. 1, pp. 82–87, 2011. The authors declare that there is no conﬂict of interest regarding the publication of this article nor are there any disclaimers present at this time. Acknowledgments All funding for the publication of this article was funded by the author’s supporting institution, CarePoint Health Bayonne Medical Center. References  R. L. Siegel, K. D. Miller, and A. Jemal, “Cancer statistics, 2018,” CA: a Cancer Journal for Clinicians, vol. 68, no. 1, pp. 7–30, 2018.  P. Strati, J. H. Uhm, T. J. Kaufmann et al., “Prevalence and characteristics of central nervous system involvement by chronic lymphocytic leukemia,” Haematologica, vol. 101, no. 4, pp. 458–465, 2016.  A. A. Moazzam, J. Drappatz, R. Y. Kim, and S. 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Published: Jan 11, 2020
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