A CASE OF EVANS SYNDROME IN A SURVIVOR OF STAGE IV HODGKIN'S LYMPHOMA

Abstract

TOPIC: Critical Care TYPE: Medical Student/Resident Case Reports INTRODUCTION: Evans syndrome (ES) is a rare disease characterized by a combination of autoimmune hemolytic anemia, immune thrombocytopenia and/or immune neutropenia. Not much is known about the exact cause of ES, but the leading hypothesis is that it's an immune system disorder. Most patients diagnosed with this disease are fifty year old females. Associated symptoms are fatigue, dyspnea, petechiae and recurring infections. It is important to distinguish primary ES from secondary ES because the etiologies and treatment regimens options are different. Since secondary ES is associated with malignancies, immunodeficiencies and other autoimmune conditions, it is found to have a higher mortality rate than primary ES. Here, we present a case of ES in a survivor of stage IV Hodgkin's lymphoma (HL). CASE PRESENTATION: A 42 year-old male with a pmh of stage IV HL was admitted for fatigue and mucosal bleeding for the past several days. Labs were significant for a hemoglobin of 8.9 g/dL and platelet count of 3x10^3 cell/µl for which the patient was given 1u of platelets. Platelet count increased to 78 x10^3 cell/µl after the transfusion, but then decreased to 5x10^3 cell/µl in less than 24 hours (Figure 1). CT chest was negative. Peripheral smear showed dacrocytes and basophilic stippling. Despite multiple blood product transfusions, the patient's CBC did not improve. Bone marrow aspirate and biopsy revealed early and abnormal megakaryocytes. Fibrinogen and Pi-LINK antigen levels were normal. FISH, flow cytometry and serum electrophoresis were negative. Direct Coombs test was positive. Patient was started on prednisone, romiplostim and rituximab, which improved his platelet count to 150x10^3 cell/µl. Patient was finally diagnosed with ES. DISCUSSION: The degree of anemia, thrombocytopenia and neutropenia will dictate the type of symptoms experienced by ES patients. Since most of them will only present themselves to the hospital once they experience moderate symptoms, most will require a SDU/ICU level of care. Approximately 10% of all ES cases are caused by malignancies. NHL and CLL are the most common malignancies associated with this disorder, but there have also been very few cases of HL patients developing ES. Other hematological conditions such as TTP, PNH, MDS or aplastic anemia must be ruled-out first as they can present similarly to ES. A nationwide survey indicated that the mean survival rate for secondary ES is 1.7 years. First-line therapy includes prednisone, romiplostim and rituximab . Other therapies include bone marrow transplants and splenectomy. CONCLUSIONS: ES in HL patients is exceedingly uncommon. Even though there is very little known about this disorder, it is important to keep it in mind whenever a patient presents with an unexplained bicytopenia because it can lead to an earlier diagnosis. A better understanding of this syndrome can improve treatment modalities and survival outcomes. REFERENCE #1: Jaime-Pérez JC, Aguilar-Calderón PE, Salazar-Cavazos L, Gómez-Almaguer D. Evans syndrome: clinical perspectives, biological insights and treatment modalities. J Blood Med. 2018;9:171-184. Published 2018 Oct 10. doi:10.2147/JBM.S176144 REFERENCE #2: Michel M, Chanet V, Dechartres A, et al. The spectrum of Evans syndrome in adults: new insight into the disease based on the analysis of 68 cases. Blood. 2009;114(15):3167-3172. doi:10.1182/blood-2009-04-215368 REFERENCE #3: Audia S, Grienay N, Mounier M, Michel M, Bonnotte B. Evans' Syndrome: From Diagnosis to Treatment. J Clin Med. 2020;9(12):3851. Published 2020 Nov 27. doi:10.3390/jcm9123851 DISCLOSURES: No relevant relationships by Mariam Agladze, source=Web Response No relevant relationships by Jonathan Ariyaratnam, source=Web Response No relevant relationships by HOWARD CHUNG, source=Web Response No relevant relationships by Vikram Sumbly, source=Web Response No relevant relationships by Theo Trandafirescu, source=Web Response