Denise da Cunha Pasqualin

A life-threatening case of TAFRO syndrome with dramatic response to tocilizumab, rituximab, and pulse steroids: The first case report in Latin America

Rationale: This is the report of the first case of TAFRO syndrome (Thrombocytopenia, Anasarca, myelofibrosis, Renal dysfunction, Organomegaly) in Latin America. Patient concerns: The patient was a 61-year-old white woman of Ashkenazi Jewish descent, who presented with a history of 8 days of nausea, vomiting, and fever; severe pitting edema in both legs, ascites, splenomegaly, and palpable axillary lymph nodes. Diagnoses: Abdominal computed tomography (CT) showed bilateral pleural effusion and retroperitoneal lymph node enlargement. Interventions: Anasarca and worsening of renal function led to admission to the intensive care unit (ICU) with multiple organ failure, requiring mechanical ventilation, vasopressor medications, and continuous renal replacement therapy (CRRT). Diagnosis of TAFRO syndrome was made on day 18 after admission, based on clinical findings and results of bone marrow and lymph node biopsies. She was treated with methylprednisolone, tocilizumab, and rituximab. One week after the first tocilizumab dose, she had dramatic improvements in respiratory and hemodynamic status, and was weaned from ventilator support and vasopressor medications. Outcomes: After 2 weeks of therapy, CRRT was switched to intermittent hemodialysis. On day 46, the patient was discharged from the ICU to the general ward, and 3 months after admission, she went home. Lessons: Provided the interleukin-6 measurement is available, this approach is suggested in cases of TAFRO syndrome, in order to customize the treatment.

Normalization of lymphocyte count after high ablative dose of I-131 in a patient with chronic lymphoid leukemia and secondary papillary carcinoma of the thyroid. Case report

ABSTRACT The authors report the case of a 70-year-old male patient with chronic lymphoid leukemia who presented subsequently a papillary carcinoma of the thyroid with metastases to regional lymph nodes. The patient was treated with surgical thyroidectomy with regional and cervical lymph node excision and radioiodine therapy (I-131). The protocolar control scintigraphy 4 days after the radioactive dose showed I-131 uptake in both axillae and even in the inguinal regions. PET/CT showed faint FDG-F-18 uptake in one lymph node of the left axilla. An ultrasound guided fine needle biopsy of this lymph node identified by I-131 SPECT/CT and FDG-F-18 PET/CT revealed lymphoma cells and was negative for thyroid tissue and thyroglobulin content. The sequential blood counts done routinely after radiation treatment showed a marked fall until return to normal values of leucocytes and lymphocytes (absolute and relative), which were still normal in the last control 19 months after the radioiodine administration. Chest computed tomography showed a decrease in size of axillary and paraaortic lymph nodes. By immunohistochemistry, cells of the lymphoid B lineage decreased from 52% before radioiodine therapy to 5% after the procedure. The authors speculate about a possible sodium iodide symporter expression by the cells of this lymphoma, similar to some other non-thyroid tumors, such as breast cancer cells.

Endoscopy Biopsy in Different Sites for the Diagnosis of Lower and Upper Gastrointestinal Graft-Versus-Host Disease

Cell dose is a major criterion for cord blood unit (CBU) selection for allogeneic stem cell transplantation (allo-SCT). The aim of this study was the characterization of CBU cellular composition after thaw, and comparisonwith corresponding values at cryopreservation as reported by cord blood (CB) banks. The study included 87 CBUs, that were thawed for infusion in the context of single (n1⁄43) or dual-unit (n1⁄442) allo-SCT in adults with hematologic malignancies, from 8/2006 to 6/2013. Upon thawing, the cryoprotective solution (DMSO 10%) was either removed by centrifugation/washing (38 CBUs) or diluted in a less hypertonic solution of Dextran 40/Human Albumin 2.5% (49 CBUs). Total nucleated cells (TNC) were measured with a hematology analyzer, while enumeration of CD34+ stem cells was performed by singleplatform flow cytometry, according to ISHAGE guidelines. In 49 units, TNC and CD34+ cell viability was evaluated by addition of 7-AAD dye and sequential Boolean gating strategy. TNC counts after thawing were lower compared to their values at freezing (Wilcoxon test, p <10-4), and the difference was more pronounced in the units that were washed prior to infusion (Tables 1 and 2). Total cell viability was low (mean value, 42.6%), but this was mainly due to neutrophils. Regarding CD34+ cells, there was a significant difference between absolute counts at cryopreservation and at thaw (p<10-4). Despite reduction postthaw, the counts of both TNC and CD34+ cells did correlate with the corresponding values at cryopreservation by Spearman’s analysis. Of note, washing seemed slightly advantageous in terms of CD34+ recovery (Tables 1 and 2). CD34+ cells retained high viability after thaw, with 90% of CBUs (44 out of 49 tested) demonstrating CD34+ viability 80%. Viability of <50% was noticed in only one CBU that failed to engraft. In conclusion, CB cellular content and especially the CD34+ cell count is frequently shown to be inferior at thaw compared to cryopreservation. This probably reflects both the lack of standardization of CD34+ cell measurement and the effect of thawing procedure. Therefore, CD34+ cell viability may be a more meaningful marker for determining CBU quality. GVH/GVL