Posters
Presentation Type
Poster
Discipline Track
Other
Abstract Type
Case Report
Abstract
Background: Hemophagocytic syndrome is a rare and potentially fatal disorder characterized by abnormal immune activation leading to multiorgan dysfunction. Although genetic mutation is the most common cause in the pediatric population, adults often present with HPS secondary to infection, autoimmune disease or malignancy. Hodgkin’s lymphoma is a type of lymphoma characterized by Reed Sternberg cells. HL can cause various complications including hemophagocytic syndrome. This is a case of a 22-year-old male with incidental pancytopenia and jaundice, found to have Hodgkin’s lymphoma and hemophagocytic syndrome.
Case Presentation: A 22-year-old male with no significant past medical history presented to the Emergency Department with fatigue, malaise, fevers and jaundice for three weeks prior to arrival.
Laboratory investigations showed a hemoglobin of 12.1, WBC 0.88, platelets 49,000. Other pertinent lab findings included creatinine 2.21, bilirubin 11.34, ALT 522, AST 751, alkaline phosphatase 1166. Additional investigations revealed incidence of hemophagocytic syndrome, including ferritin 43034, LDH 1400 and triglycerides 405.
A non-contrast CT Abdomen and Pelvis was performed to evaluate for underlying malignancy which showed the liver and spleen to be enlarged, measuring 23 cm and 19 cm respectively and nonspecific, moderate amount of retroperitoneal adenopathy. An excisional retroperitoneal lymph node biopsy demonstrated Hodgkin’s lymphoma with presence of Reed Sternberg cells.
The patient was started on pulse Dexamethasone and Gemcitabine followed by weekly Cisplatin. He was not able to be treated with the standard therapy for Hodgkin’s lymphoma secondary to renal and liver dysfunction. He then received chemotherapy with Doxorubicin and Dacarbazine. The renal and liver function continued to improve and then received two cycles of ABVD.
Follow up PET scan showed residual 3 cm retroperitoneal lymph nodes around the left iliac artery with a maximum SUV of 2.8 suggestive of posttreatment effect. He then completed six cycles of chemotherapy. Another PET scan thereafter showed complete metabolic response. He responded well to treatment with improvement in pancytopenia, jaundice and resolution of hepatosplenomegaly.
Conclusion: HPS is a rare but serious complication of Hodgkin’s lymphoma with an incidence ranging from 4 to 10%. The systemic inflammation that characterizes the disease is the result of inappropriate and dysregulated activation of natural killer (NK) cells, CD8+ cytotoxic T-cells, and macrophages. The disease is classified as either primary or secondary. Patients with primary disease present early in childhood, whereas those with secondary disease present as adults with an associated acute illness, most commonly sepsis or a hematologic malignancy.
Hemophagocytic syndrome can occur at any stage of the disease and its clinical presentation can overlap with HL, making early diagnosis challenging. The pathogenesis of HPS in HL is not fully understood, but it is believed to be related to the cytokine storm that occurs in response to the malignant cells.
The treatment of Hodgkin’s lymphoma associated hemophagocytic syndrome involves the management of both HL and HPS. Chemotherapy is the mainstay of treatment for HL and high doses of steroids are used to suppress the immune response in HPS. Supportive care including blood transfusions and prophylactic antibiotics is also essential.
Academic/Professional Position
Resident
Mentor/PI Department
Internal Medicine
Recommended Citation
Mehta, Radhika and Akhtar, Bareera, "Hemophagocytic Syndrome" (2024). Research Symposium. 8.
https://scholarworks.utrgv.edu/somrs/2023/posters/8
Included in
Hemophagocytic Syndrome
Background: Hemophagocytic syndrome is a rare and potentially fatal disorder characterized by abnormal immune activation leading to multiorgan dysfunction. Although genetic mutation is the most common cause in the pediatric population, adults often present with HPS secondary to infection, autoimmune disease or malignancy. Hodgkin’s lymphoma is a type of lymphoma characterized by Reed Sternberg cells. HL can cause various complications including hemophagocytic syndrome. This is a case of a 22-year-old male with incidental pancytopenia and jaundice, found to have Hodgkin’s lymphoma and hemophagocytic syndrome.
Case Presentation: A 22-year-old male with no significant past medical history presented to the Emergency Department with fatigue, malaise, fevers and jaundice for three weeks prior to arrival.
Laboratory investigations showed a hemoglobin of 12.1, WBC 0.88, platelets 49,000. Other pertinent lab findings included creatinine 2.21, bilirubin 11.34, ALT 522, AST 751, alkaline phosphatase 1166. Additional investigations revealed incidence of hemophagocytic syndrome, including ferritin 43034, LDH 1400 and triglycerides 405.
A non-contrast CT Abdomen and Pelvis was performed to evaluate for underlying malignancy which showed the liver and spleen to be enlarged, measuring 23 cm and 19 cm respectively and nonspecific, moderate amount of retroperitoneal adenopathy. An excisional retroperitoneal lymph node biopsy demonstrated Hodgkin’s lymphoma with presence of Reed Sternberg cells.
The patient was started on pulse Dexamethasone and Gemcitabine followed by weekly Cisplatin. He was not able to be treated with the standard therapy for Hodgkin’s lymphoma secondary to renal and liver dysfunction. He then received chemotherapy with Doxorubicin and Dacarbazine. The renal and liver function continued to improve and then received two cycles of ABVD.
Follow up PET scan showed residual 3 cm retroperitoneal lymph nodes around the left iliac artery with a maximum SUV of 2.8 suggestive of posttreatment effect. He then completed six cycles of chemotherapy. Another PET scan thereafter showed complete metabolic response. He responded well to treatment with improvement in pancytopenia, jaundice and resolution of hepatosplenomegaly.
Conclusion: HPS is a rare but serious complication of Hodgkin’s lymphoma with an incidence ranging from 4 to 10%. The systemic inflammation that characterizes the disease is the result of inappropriate and dysregulated activation of natural killer (NK) cells, CD8+ cytotoxic T-cells, and macrophages. The disease is classified as either primary or secondary. Patients with primary disease present early in childhood, whereas those with secondary disease present as adults with an associated acute illness, most commonly sepsis or a hematologic malignancy.
Hemophagocytic syndrome can occur at any stage of the disease and its clinical presentation can overlap with HL, making early diagnosis challenging. The pathogenesis of HPS in HL is not fully understood, but it is believed to be related to the cytokine storm that occurs in response to the malignant cells.
The treatment of Hodgkin’s lymphoma associated hemophagocytic syndrome involves the management of both HL and HPS. Chemotherapy is the mainstay of treatment for HL and high doses of steroids are used to suppress the immune response in HPS. Supportive care including blood transfusions and prophylactic antibiotics is also essential.