Abstract | November 16, 2023

High-Grade B Cell Lymphoma in the Setting of Janus Kinase (JAK) Inhibitors and Post-Stem Cell Transplantation

Sunpil Hwang, MD, Internal Medicine Resident PGY 2, North Alabama Medical Center, Florence, AL

Sangeetha Issac, MD, Internal Medicine, Fellow of hematology/oncology, ECU Health Medical Center, Greenville, NC; Brett Barlow, MD, Internal Medicine, Attending, Clearview Cancer Center, Florence, AL

Learning Objectives

  1. Upon completion of this lecture, learners should be better prepared to be wary of the heightened risk of developing lymphoproliferative disorders within the patient groups who underwent hematopoietic cell transplantation and to diligently monitor and examine the patients on a regular basis. It is known that individuals who underwent hematopoietic cell transplantation are at an increased risk of developing lymphoproliferative disorders, with an incidence estimated to be approximately 1-2%.
  2. Upon completion of this lecture, learners should be better prepared to proactively monitor patients on JAK 1/2 inhibitors as it can increase the risk of secondary cancer. Several reports have demonstrated an increased incidence of aggressive B-cell lymphoma among patients receiving JAK 1/2 inhibitors, as illustrated in this case. Although there is no official incidence rate of secondary lymphoma among these patients, several cohort studies have shown a significant increase in the risk, with up to a 15-fold increase in risk.

Introduction:
Lymphoma is associated with various risk factors, including immune deficiency. Hematopoietic stem cell transplantation (HSCT) is also known to be a risk factor for lymphoproliferative disorders. Furthermore, recent studies have reported a potential link between using Janus kinase (JAK) 1/2 inhibitors and an increased risk of aggressive B-cell lymphoma. The immunosuppressive effects of JAK 1/2 inhibitors have been suggested to contribute to the elevated risk of developing aggressive B-cell lymphoma.

Case presentation:
A 67-year-old female with a medical history of JAK2 positive myelofibrosis on ruxolitinib, status post failed stem cell transplantation 2 years ago, presented to the emergency room with worsening right lower quadrant pain over the last 2-3 months after the colonoscopy. The patient denied fever, nausea, vomiting, bowel habit changes, or weight loss. On presentation, her vital signs were normal. Physical examination was unremarkable except for right lower quadrant tenderness.

The initial CBC revealed moderate anemia, with a hemoglobin level of 8.1, and thrombocytopenia, with a platelet count of 29. The CMP yielded unremarkable results. An abdominal CT at another facility taken a few days prior to admission revealed a large necrotic mass arising from the cecum, measuring 7.3 x 7.7 x 8.2 cm. The report suggested the possibility of a large necrotic tumor. However, in light of a recent colonoscopy that showed only diverticulosis and undetectable carcinoembryonic antigen, she was started with a trial of antibiotics-vancomycin, levofloxacin, and metronidazole-for suspected abscess.

Following three days of antibiotic treatment, a follow-up CT scan with contrast was performed, which did not show any improvement in the size of the mass. Instead, it revealed a large necrotic cecal mass that extended into the right iliopsoas muscle, encircling the mid-right ureter and the right common iliac artery bifurcation. These findings were consistent with a neoplasm.

Final Diagnosis:
The repeated colonoscopy revealed an infiltrative and ulcerated large mass in the ascending colon. Biopsy from the lesion confirmed high-grade B cell lymphoma.

Management and follow-up:
The patient initiated chemotherapy with R-CVP (Rituximab, Cyclophosphamide, Vincristine, and Prednisolone) and was subsequently discharged. Ruxolitinib was discontinued. She continued to follow hematology/oncology for chemotherapy as an outpatient.

References and Resources

  1. Abbas, F., El Kossi, M., Shaheen, I. S., Sharma, A., & Halawa, A. (2020). Post-transplantation lymphoproliferative disorders: Current concepts and future therapeutic approaches. World journal of transplantation, 10(2), 29–46. https://doi.org/10.5500/wjt.v10.i2.29
  2. Landgren, O., Gilbert, E. S., Rizzo, J. D., Socié, G., Banks, P. M., Sobocinski, K. A., Horowitz, M. M., Jaffe, E. S., Kingma, D. W., Travis, L. B., Flowers, M. E., Martin, P. J., Deeg, H. J., & Curtis, R. E. (2009). Risk factors for lymphoproliferative disorders after allogeneic hematopoietic cell transplantation. Blood, 113(20), 4992-5001. https://doi.org/10.1182/blood-2008-09-178046
  3. Porpaczy, E., Tripolt, S., Hoelbl-Kovacic, A., Gisslinger, B., Bago-Horvath, Z., Casanova-Hevia, E., Clappier, E., Decker, T., Fajmann, S., Fux, D. A., Greiner, G., Gueltekin, S., Heller, G., Herkner, H., Hoermann, G., Kiladjian, J.-J., Kolbe, T., Kornauth, C., Krauth, M.-T., Kralovics, R., … Gisslinger, H. (2018). Aggressive B-cell lymphomas in patients with myelofibrosis receiving JAK1/2 inhibitor therapy. Blood, 132(7), 694-706. https://doi.org/10.1182/blood-2017-10-810739
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