¹Atteia Al Houri,  Mohammed Alnuwaysir, Dr. Mouayd Alshakhal, ¹Dr. Mohammed Alnuwaysir,
¹Mouayd Alshakhal

Background(s): Evans syndrome (ES) is a rare syndrome, it is characterized by the presence of autoimmune hemolytic anemia, immune thrombocytopenia, and/or immune neutropenia of unknown etiology. It occurs mainly in children, usually due to primary immunodeficiencies or autoimmune lymphoproliferative syndrome. As the hematological abnormalities are an important diagnostic criterion for Systemic Lupus Erythematosus (SLE), we present a patient diagnosed with ES with underlying SLE on initial presentation.

Method(s): A 47 years old, Saudi female, with a past medical background of sickle cell trait, hypothyroidism on levothyroxine, pancytopenia followed with a hematologist and managed her as a case of myelodysplasia for 6 years, refractory to recurrent blood, and platelets transfusion, bone marrow biopsy was done 3 times in the past showed hypercellular reactive bone marrow with trilineage hematopoiesis no evidence of myelodysplasia . She recently encountered a COVID-19 infection 1 month before presentation. The patient presented to our hospital with a long history of generalized weakness, fatigue, dizziness, and exertional dyspnea. Her symptoms gradually worsened over time  that she sever fatigue and dizziness on standing  . She reported easily bruising on her extremities, recurrent gum bleeding after brushing her teeth, epistaxis, and menorrhagia which resulted in recurrent iron deficiency anemia.

Result(s): workup upon this presentation showed hypochromic microcytic anemia with Hemoglobin 5.1 g/dL, total leukocyte counts 1.9 X103/uL [4-10], red blood cells 2.32  [3.8-4.8], and platelets 2 X103/uL [150-430], with neutropenia (Neutrophil count 0.96 X10^3/uL) [1.5-6] and lymphopenia (Lymphocyte count 0.46 X10^3/uL) [1.3-2.9] in the differential count. Positive direct Coombs test and peripheral blood film showed moderate leukopenia, slight hypochromic microcytosis, slight teardrop cells, target cells, and thrombocytopenia. Her reticulocyte count was 6.29% [3-6], and her lactate dehydrogenase (LDH) 382 IU/mL [135-225]. Further labs showed low C3 (0.57 g/L) normal C4 (0.18 g/L), ANA 1:320 nucleolar, and anti-DNA ABs (57 IU/ml), Anti SSB (LA): Positive. Bone Marrow aspiration & trephine biopsy: Reactive cellular marrow and intact trilineage hematopoiesis with no evidence of dysplasia or infiltration.

Conclusion(s): Given the patient’s clinical presentation with the above laboratory findings, we diagnosed the patient as a case of Evan syndrome 2ry to SLE, as the patient has hemolytic anemia, immune thrombocytopenia, lymphopenia and neutropenia. She received IV pulse steroid for 3 days, then shifted to oral tapering down steroid, Intravenous immune globulin (IVIG) for 5 days, and eltrombopag olamine. She showed a marked improvement clinically, as well as continued improvement in her anemia and thrombocytopenia after starting her on treatment