Cryoglobulinemia Secondary to Sjögren Syndrome after Kidney Transplant

Introduction: The most frequent autoimmune disease that underlies non HCV-induced cryoglobulinaemia is Sjögren syndrome. Among the most common findings of Sjögren's syndrome from a hematological point of view are cytopenias: anemia, leukopenia, thrombocytopenia and also cryoglobulinemia and lymphoma. In post-kidney transplant patients, it is less common to find Sjögren's syndrome. Monoclonal gammopathy of undetermined significance (MGUS) has been observed in 20% of Sjogren's patients when serum immunoelectrophoresis is routinely performed. About 5% of patients with this abnormality may progress to develop lymphoma or another hematologic malignancy Case Description: A 23-year-old female patient received a kidney transplant from a related living donor in July 2023. In September 2023, patient presented with difficult pancytopenia management, so autoimmune tests are performed and AntiRo >200 is evident, Sjögren's syndrome is diagnosed and a biopsy of minor salivary glands is performed where periductal lymphocytic sialadenitis is observed (Figure 1), patient persists with leukocyte count as follows: WBC 3060 Neutrophils 84 %, Hemoglobin in 9.16 g/dL, platelets in 126,000, peripheral blood smear was performed within normal limits, patient with refractory cytopenias for which cryoglobulins were performed which in 18%, rheumatoid factor 12.5 (normal), IgG 848.05, electrophoresis and negative protein immunofixation, creatinine at 1.87mg/dl, 24-hour urine protein at 186, refractory cytopenia is considered a manifestation of cryoglobulinemia type I secondary to Sjögren's syndrome, so it was decided to admit the patient to start treatment with plasmapheresis (5 sessions) and rituximab (1gr) for refractory presentation of cytopenias. Discussion: Our patient presented refractory cytopenias secondary to cryoglobulinemia due to Sjögren's syndrome, which is a common cause not associated with HCV but a common cause of autoimmune disease. However, it is not common in post-transplant patients and currently our patient has no renal manifestations of the disease, there is no evidence of glomerulonephritis and the graft is functioning properly.