Objective: Microangiopathic hemolytic anemia (MAHA) can be observed as a paraneoplastic syndrome (PS) in certain tumors. MAHA related signet ring cell carcinoma (SRCC) of an unknown origin is very infrequent. Herein we present a SRCC case presented with refractory acquired thrombotic thrombocytopenic purpura (TTP).
Case report: 35 years old men applied to emergency service with fatigue and headache on January 2020. In his anamnesis he had a history of alcoholic pancreatitis. His physical examination was normal except the neurological symptoms which are temporary loss of consciousness and disorientation. His laboratory tests resulted as white blood cell 9020/μL, hemoglobin 3.5g/dL, platelet 18,000/μL, MCV 110.7fl, urea 58mg/dL, creatinine 0.84mg/dL, AST 68u/L, ALT 33u/L, indirect bilirubin 1.88mg/dL, LDH 2257u/L, reticulocyte 0.1, haptoglobulin <8mg/dL, INR 1.42, Prothrombin time 13.2, fibrinogen 184mg/dL, coombs negative. He had consulted to our clinic with bicytopenia and hemolysis. Schistocytes, micro-spherocytes and thrombocytopenia were observed in his blood smear. Microangiopathic hemolytic anemia was present and he was considered as thrombotic thrombocytopenic purpura. Plasma exchange treatment was initiated however he was refractory to this treatment. He had epistaxis and blurred vision during the follow-up. Superficial hemorrhages on the edges of the optic disc and roth spots were detected. Pain had emerged in his right arm. Doppler ultrasonography revealed the occlusion of cephalic vein with non-recanalized thrombus in the subacute process from the antecubital level at the forearm level. Thorax and abdomen computerized tomography (CT) resulted as liver 220cm, spleen 14cm, minimal pleural effusion, thickening of minor curvature in stomach corpus with hepatogastric and paraceliac lymphadenopathy. As a result of CT endoscopic examination was planned. Bone marrow investigation by our clinic resulted as the metastasis of adenocarcinoma. Ulcerations and necrosis was observed by gastric endoscopy procedure. Biopsy was taken during endoscopic intervention which resulted as signet ring cell carcinoma. He was transferred to oncology clinic for his treatment. Unfortunately he died in one month after his transfer.
Conclusion: Only 40% of TTP cases have the complete pentad and in 75% of the cases there is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and neurological findings. In our case there was no acute kidney failure, however all the other features favored TTP, and diagnosis was made without the kidney failure. MAHA may be seen as a PS in some tumors, especially gastric cancers. Tumor related MAHA is generally accompanied by bone marrow (BM) metastases. As a result, BM investigation may be used as the main diagnostic method to find the underlying cancer. Total plasma exchange is usually performed in the treatment of cancer-associated TTP, however fewer than 20% of the cases respond to plasma exchange. Likely, our case did not respond to plasma exchange treatment either. The clinical course of cases with tumor related MAHA is usually poor, and these cases are usually refractory to plasma exchange treatment. In conclusion, physicians should suspect a malignancy and BM involvement when faced with a case of refractory TTP.
