Abstract: PUB509
Cryoglobulinemia Secondary to Sjögren Syndrome after Kidney Transplant
Session Information
Category: Transplantation
- 2102 Transplantation: Clinical
Author
- Chavez, Ada Noemi, Hospital General San Juan de Dios, Ciudad de Guatemala, Guatemala
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.