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Home , Alemtuzumab, Brentuximab vedotin

Hematology Reports 2016; volume 8:6593

Brentuximab vedotin therapy tive tumor cells, and was effective in diminish- ing the cutaneous lesions seen in our patient Correspondence: Jordan Senchak, Drexel for cutaneous lesions in after relapse. University College of Medicine, Philadelphia, T-prolymphocytic : 2510 Maryland Road, Willowood Building Suite a case report 175, Willow Grove, PA 19090, USA. Tel: +1.724.714.2337. E-mail: [email protected]

1 2 Case Report Jordan Senchak, Peter Pickens Key words: T-prolymphocytic leukemia; brentux- 1Drexel University College of Medicine, imab vedotin; . We report an 88-year-old man who presented Philadelphia; 2Department of with weight loss, paraneoplastic sweats, Hematology/Oncology, Abington Contributions: JS, collected data and authored headaches and over a 3-4 month the paper. PP, reviewed and edited the paper. Memorial Hospital, Abington, PA, USA course in April 2014. Physical examination was remarkable for pallor and peripheral lym- Conflict of interest: the authors declare no poten- phadenopathy with an absence of tial conflict of interest. splenomegaly and any cutaneous involvement. Abstract Bone marrow biopsy showed 45% cellularity Received for publication: 10 May 2016. with 30-35% T-cells that coexpressed CD4 and Accepted for publication: 30 August 2016. CD8 and were positive for CD52. Flow cytome- We present an 88-year-old male with simul- This work is licensed under a Creative Commons taneous T-cell prolymphocytic leukemia and try detected 40% abnormal T cells that Attribution-NonCommercial 4.0 International stable smoldering myeloma with excellent ini- expressed CD2, cytoplasmic but not surface License (CC BY-NC 4.0). tial response to three months of alemtuzumab. CD3, CD4, CD5, CD7, and CD8. FISH analysis The patient relapsed at twelve months with did not detect a TcR alpha/delta rearrange- ©Copyright J. Senchak and P. Pickens, 2016 severe cutaneous disease. Biopsy of a repre- ment, but did identify a +14q32.33 in an aver- Licensee PAGEPress, Italy sentative plaque demonstrated CD30 positivity age of 28% of the nuclei, which raised the pos- Hematology Reports 2016; 8:6593 in rare malignant . The patient sibility of an upstream abnormality in the T- doi:10.4081/hr.2016.6593 demonstrated no response to reintroduction cell leukemia/-1A (TCL1A)/TCL1B with a full course of alemtuzumab. He was gene region, which is common in T-PL. therefore treated with , Karyotyping showed a gain of chromosome 8 that persisted for four months (Figures 1 and resulting in partial remission of skin involve- and deletion of 11q, classic features of T-PL. 2). When seen in February 2016 with progres- ment that persisted for three months. Immunostains showed 10% kappa-restricted sion of severe cutaneous involvement he com- plasma cells, and flow cytometry detected a menced treatment with Pralatrexate to which monoclonal kappa plasma cell population. he has currently demonstrated partial These findings were consistent with a plasma response of skin disease. Throughout his Introduction cell , and in the absence of myeloma- course, he has never developed progression to related organ or tissue damage represented avert multiple myeloma. T-cell prolymphocytic (T-PL) is a rare, smoldering myeloma. Myeloma FISH analysis mature, and aggressive T-cell neoplasm involv- did not detect abnormalities that are typically ing multiple organ systems, including blood, seen in malignant plasma cells. CBC showed a bone marrow, spleen, and lymph nodes. T-PL leukocytosis with an absolute Discussion comprises 2-5% of mature lymphocytic count 6400, count of 20.0, leukemia in adult patients.1 Older adults are 33.8% lymphs, 6% monos, 60.2% PMNs, and most often affected, with a median age of 61.2 low hemoglobin of 10.2. CT scan demonstrated We report a case of a patient with T-PL with Patients most often present with extensive mesenteric lymphadenopathy and rare CD30 expression that demonstrated a par- hepatosplenomegaly, lymphocytosis, and gen- non-bulky axillary and mediastinal lym- tial response to treatment with a monoclonal eralized lymphadenopathy. Cutaneous mani- phadenopathy. Liver and spleen were normal directed against CD30 in malignant festations in T-PL are common, consisting of in size. Skeletal survey did not demonstrate lymphocytes. The skin lesions initially rash, papules, purpura, and ulcers.3 osteolysis. Treatment with 30 mg alemtuzum- responded to treatment with CD52 specific Presentation of the skin lesions can range ab was started three times a week for 12 alemtuzumab, as this is the first-line treat- from initial diagnosis up to two years post weeks. Following treatment with alemtuzum- ment for T-PL. Such treatment associates with diagnosis.3 Alemtuzumab is the first line ther- ab, he demonstrated resolution of constitu- an 80% remission rate, although remission 6-7 apy for T-PL, as most cases are CD52 positive. tional symptoms, resolution of lymphocytosis, tends to be brief. BV is most often used in Despite alemtuzumab efficacy in treatment of and improvement of anemia. lymphoid malignancies that more characteris- T-PL, median overall survival is only one year, In July of 2015, roughly 15 months after tically express CD30, such as Hodgkin lym- with less than a 10% survival at 5 years, and a remission, he relapsed with open, weeping phoma and systemic anaplastic large cell lym- relapse rate of nearly 100%.4-7 Although our sores on his back. Biopsy demonstrated posi- phoma.8 The most common side effect is patient responded to alemtuzumab, he tivity for CD2, CD3, CD5, CD7, CD25 and CD30 peripheral neuropathy and nausea/vomiting.9 relapsed after roughly twelve months with in rare malignant T-cells. He was restarted on In our patient, no neuropathy was noted and severe cutaneous involvement, and was unre- another 12-week course of alemtuzumab with treatment was well tolerated. Our case shows sponsive to repeat therapy. Brentuximab little improvement in skin disease. In that CD30 with BV has activi- vedotin (BV) is an antibody-conjugate consist- December, he was started on anti-CD30 treat- ty in recurrent, alemtuzumab resistant T-PL. ing of a chimeric immunoglobulin specific for ment with BV due to the rare CD30 positive Physicians caring for patients with T-PL should CD30, with cytotoxic auristatin E, and a pro- cells found in the excisional skin biopsy. He consider immunostaining these malignant T tease covalent linker.5 This treatment provides demonstrated a partial remission of cutaneous cells for CD30 expression and consider BV sal- efficient, targeted therapy against CD30 posi- lymphoma following three treatments of BV vage treatment if cells express positivity.

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Figure 1. Pre brentuximab vedotin treatment, December 2015. Figure 2. Post brentuximab vedotin treatment, February 2015.

2014;38:1468-83. Clin Oncol 1997; 15:2667-72. References 4. Dearden CE, Khot A, Else M, et al. 7. Dearden C. How I treat prolymphocytic Alemtuzumab therapy in T-cell prolympho- leukemia. Blood 2012;120:538-51. 1. Robak T, Robak P. Current treatment cytic leukemia: comparing efficacy in a 8. Garnock-Jones, K. Brentuximab vedotin: a options in prolymphocytic leukemia. Med series treated intravenously and a study review of its use in patients with hodgkin piloting the subcutaneous route. Blood Sci Monit 2007;13:69-80. lymphoma and systemic anaplastic large 2. Dearden C. Management of prolymphocyt- 2011;118:5799-802. cell lymphoma following previous treat- ic leukemia. Hematology Am Soc Hematol 5. Hasanali Z, Bikramajit S, Stuart A, et al. ment failure. Drugs 2013;73:371-81. Educ Program 2015;2015:361-7. Epigenetic therapy overcomes treatment 3. Hsi AC, Robird DH, Luo J, et al. T-cell pro- resistance in prolymphpocytic 9. Duvic M, Tetzlaff MT, Gangar P, et al. lymphocytic leukemia frequently shows leukemia. Sci Trans Med 2015;7:192. Results of a phase II trial of brentuximab cutaneous involvement and is associated 6. Pawson R, Dyer MJ, Barge R, et al. vedotin for CD30+ cutaneous T-cell lym- with gains of MYC, loss of ATM, and TCL1A Treatment of T-cell prolymphocytic phoma and lymphomatoid papulosis. J Clin rearrangement. Am J Surg Path leukemia with human CD52 antibody. J Oncol 2015;33:3759-65.

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