Objective: Cancer-associated microangiopathic hemolytic anemia (MAHA) is a rare but serious condition that is encountered in patients diagnosed with a malignancy. We describe a case of signet-ring cell carcinoma with a very rare presentation, namely a laboratory and clinical picture of MAHA, who demonstrated an effective thrombocyte level in response to therapeutic plasma exchange (TPE) therapy that was administered during the diagnostic period.
Case report: A 42-year-old male patient was referred to our hospital by an external center due to the complaint of recurrent epistaxis in the recent days, leukocytosis, anemia, and thrombocytopenia detected in his complete blood count. Hemogram data included the following; hemoglobin, 8.2g/dL; white blood cells, 12.9×109/L; platelet count, 25×109/L; mean corpuscular volume (MCV), 82fl. Laboratory data included the following: lactate dehydrogenase (LDH), 2826 IU/L; total bilirubin, 4.7mg/dL; indirect bilirubin, 3.4mg/dL; and a negative result on the direct antiglobulin test (Coombs). Vitamin-B12, folic acid, serum iron, and total iron-binding capacity levels, transferrin saturation, and thyroid function tests were normal. Peripheral blood smear showed fragmented erythrocytes (schistocyte), findings of erythrodysplasia, polychromasia, poikilocytosis, and in some areas, normoblasts and reticulocytosis. Reticulocyte percentage was nearly 14%. The patient was suspected of having MAHA based on these clinical, laboratory, and peripheral smear morphologic findings. Further tests were conducted in order to determine the etiology, primarily, TTP. A serum sample was collected to determine plasma ADAMTS-13 activity and therapeutic plasma exchange (TPE) was started as a treatment. Bone marrow aspiration (BMA) and biopsy (BMB) performed to examine bone marrow infiltration by hematologic and nonhematologic malignancies did not determine malignant cell infiltration. Serologies for viral infections autoantibodies were negative. A cervical-thoracic-full abdominal computed tomography (CT) scan was performed in order to detect malignancies. On abdominal CT, 1 to 2 lymphadenopathies of 15×12mm in the peripancreatic and perigastric area and pathological wall thickening (2.5cm) at the level of the gastric corpus were detected. Gastroduodenoscopy revealed an edematous, partly ulcerated lesion protruding from the mucosa that extended to the angularis from the gastric cardia. Gastric tissue biopsy report indicated poorly differentiated adenocarcinoma (signet-ring cell predominant). The case was accepted as MAHA secondary to gastric carcinoma (ADAMTS-13 activity tested earlier was within normal limits at 84%). While waiting for the results of the biopsy and the other tests, the patient underwent 14 sessions of TPE in total. Following TPE, platelet count increased from 25×109/L up to 162×109/L, fragmented erythrocyte rate in peripheral smear decreased more than 75% and other laboratory findings of hemolysis (LDH, bilirubin, etc.) significantly decreased. The patient was transferred to the medical oncology clinic for the chemotherapeutic treatment of the primary gastric carcinoma.
Conclusion: Malignancy-associated MAHA is generally linked to a poor prognosis and the optimal treatment is not known. However, there is evidence for the importance of promptly initiating an effective antineoplastic regimen and it is also noteworthy that administering therapeutic plasma exchange (TPE) therapy for the purpose of immunocomplex removal could be beneficial in patients with symptoms of bleeding and thrombosis.
