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Abstract: PO1908

Deceiving Schistocytes

Session Information

Category: Onco-Nephrology

  • 1500 Onco-Nephrology

Authors

  • Gonçalves, Catarina Abrantes, Centro Hospitalar de Setubal EPE, Setubal, Setúbal, Portugal
  • Furtado, Maria Teresa, Centro Hospitalar de Setubal EPE, Setubal, Setúbal, Portugal
  • Domingues, Patrícia Andreia da Costa, Centro Hospitalar de Setubal EPE, Setubal, Setúbal, Portugal
  • Piedade, Ana D., Centro Hospitalar de Setubal EPE, Setubal, Setúbal, Portugal
  • Natario, Ana, Centro Hospitalar de Setubal EPE, Setubal, Setúbal, Portugal
Introduction

Myelodysplastic syndrome (MDS) is a clone bone marrow disorder characterized by dyshematopoiesis, which may manifest as cytopenias and non-immune hemolytic anemia. Schistocytes are commonly associated with causes of microangiopathic hemolytic anemia (MAHA), however, schistocytosis with a high reticulocyte count in the peripheral blood smear is a rare and unusual manifestation of MDS.

Case Description

We report the case of a 63-year-old male who presented with complaints of asthenia, fatigue and malaise for the past 3 months. His previous medical history included a past of heavy smoking, arterial hypertension and grade 3 chronic kidney disease (CKD) developed after nephrectomy due to urothelial carcinoma in 2012. He also had in situ papillary urothelial carcinoma of the bladder in 2016, with a course of intra-vesical mitomycin. Vital signs were normal and physical examination was unremarkable. Blood work revealed macrocytic anemia (hemoglobin 7.2g/dL; MGV 101fL) and thrombocytopenia (77.000/μL) and peripheral blood smear demonstrated 16% schistocytes, with normal coagulation tests, lactate dehydrogenase and haptoglobin. Coombs test was negative. Renal function was stable and there was no evidence of hematoproteinuria. Inflammatory markers were negative. A diagnosis of microangiopathic anemia was assumed and the patient was started on daily plasmapheresis and steroids, while further investigation was under way. Folate and cobalamin levels were normal, anti-nuclear antibodies and HIV, hepatitis B and C serologies were negative and full-body CT scan did not show signs of occult malignancy. Levels of C3 and C4 were also within the normal range. ADAMTS-13 activity was 21%. No clinical or analytical improvement was noted after 6 sessions of plasmapheresis (platelet count nadir of 38.000/μL and persistence of schistocytosis). Bone marrow biopsy was performed and a diagnosis of refractory anemia with excess blasts was made.

Discussion

Hemolytic anemia is a common occurrence in patients with hematologic malignancies, particularly acute and chronic myeloid leukemia, but are rarely observed in MDS, with only a few cases reported in the literature. This case highlights the importance of considering a diagnosis of MDS in patients presenting with refractory cytopenias and MAHA.