A Rare Presentation Of Other Iatrogenic Immunodeficiency-Associated Lymphoproliferative Disorder, Classic Hodgkin'S Lymphoma-Type, With Hepatic Impairment Treated With Salvage Chemotherapy Of Gemcitabine

INTRODUCTION: Other iatrogenic immunodeficiency-associated lymphoproliferative disorders (Oii-LPDs) are classified as tumors of hematopoietic and lymphoid tissues occurring in those receiving immunosuppressive therapy or those with autoimmune diseases. Among this category of LPDs are classical Hodgkin lymphoma type (CHL-type) which is a rare entity. There is a lack of chemotherapy regimens for patients who present with Hodgkin lymphoma with impaired liver function due to the high side effect profile. We present an unusual case of CHL-type Oii-LPD with liver failure who was treated with salvage therapy of gemcitabine, steroids, and N-acetylcysteine (NAC). CASE DESCRIPTION/METHODS: A 39-year-old female with a history of SLE, rheumatoid arthritis on hydroxychloroquine, Raynaud’s syndrome, and alcohol abuse presented to the hospital with generalized fatigue. She arrived to the hospital from an outpatient clinic due to abnormal lab values of Hgb of 4.5g/dl and PLT count of 17,000. Her physical exam findings were significant for jaundice and cervical lymphadenopathy. On arrival to the hospital patient STAT labs indicated a T. Bili of 5.3 mg/dL, D. Bili of 2.8 mg/dL, ALK Phos of 1,270 u/L, AST 57 u/L, ALT 39 u/L, GGT 116 u/L, and LDH 220. An MRI of her abdomen was done which was significant for hepatomegaly and steatosis. A liver biopsy was deferred due to significant thrombocytopenia. Patient than had a CT scan of her chest performed which depicted large bilateral axillary lymph nodes. Axillary lymph node biopsy was positive for CD15, CD30, and PAX-5 with overall findings consistent with classical Hodgkin lymphoma and classified as other iatrogenic immunodeficiency associated lymphoproliferative disorder, classical Hodgkin lymphoma type. Given the patient’s poor hepatic function, ABVD chemotherapy was contraindicated. The patient was started on gemcitabine and methylprednisolone as salvage therapy. She did not tolerate the therapy well and developed significant thrombocytopenia leading to significant epistaxis. Family ultimately decided to pursue comfort care. DISCUSSION: Malignant infiltration of the liver with Hodgkin's disease causing acute liver failure is uncommon, but when concomitant carries a grave prognosis. Due to the small percentage of patients with aggressive lymphoma and hepatic involvement the treatment options are limited. This case study provides additional data regarding liver failure and chemotherapy regimens and alternative pathways that should be explored.Figure 1.: Bone marrow with nearly complete replacement of histiocytic infiltrates mixed with lymphocytes, plasma cells and eosinophils.Figure 2.: Immunohistochemical stain that is positive for CD30 suggesting Hodgkin lymphoma.Figure 3.: The large Hodgkin cells have single to multiple nuclei, prominent central nucleoli and abundant amounts of cytoplasm.