Idiopathic multicentric Castleman disease: a mysterious case of generalized lymphadenopathy

Castleman disease is a syndrome with significant clinico-pathological overlap between malignancy, autoimmune causes and infectious etiologies. It is a spectrum and can vary in extent from unicentric to multicentric disease with generalized lymphadenopathy, organ involvement, constitutional symptoms and cytopenias, and in severity from non-severe to severe disease with TAFRO symptoms. Idiopathic multicentric Castleman disease (iMCD) is a diagnosis of exclusion after multiple causes as per diagnostic criteria are excluded. Treatment varies between the disease severity types with anti-IL 6 antibodies for non-severe disease to cytotoxic chemotherapy agents for severe disease with TAFRO symptoms. We hereby report a case of a non-severe type of iMCD with a prolonged course and delayed arrival at the diagnosis, owing to the rarity of this condition, which stresses the need for a reduced threshold to consider MCD, early in the differential diagnosis. Interestingly there were positive auto-antibodies and elevated IgG4 levels in this patient, but applying strict criteria helps to distinguish the diagnosis

Related umbilical cord blood transplantation in patients with thalassemia and sickle cell disease.

Allogeneic bone marrow transplantation (BMT) from HLA-identical siblings is an accepted treatment for both thalassemia and sickle cell disease (SCD). However, it is associated with decided risk of both transplant-related mortality (TRM) and chronic graft-versus-host disease (GVHD). We analyzed 44 patients (median age, 5 years; range, 1-20 years) given an allogeneic related cord blood transplant for either thalassemia (n = 33) or SCD (n = 11). Thirty children were given cyclosporin A (CsA) alone as GVHD prophylaxis, 10 received CsA and methotrexate (MTX), and 4 patients received other combinations of immunosuppressive drugs. The median number of nucleated cells infused was 4.0 x 10(7)/kg (range, 1.2-10 x 10(7)/kg). No patient died and 36 of 44 children remain free of disease, with a median follow-up of 24 months (range, 4-76 months). Only one patient with SCD did not have sustained donor engraftment as compared with 7 of the 33 patients with thalassemia. Three of these 8 patients had sustained donor engraftment after BMT from the same donor. Four patients experienced grade 2 acute GVHD; only 2 of the 36 patients at risk developed limited chronic GVHD. The 2-year probability of event-free survival is 79% and 90% for patients with thalassemia and SCD, respectively. Use of MTX for GVHD prophylaxis was associated with a greater risk of treatment failure. Related CBT for hemoglobinopathies offers a good probability of success and is associated with a low risk of GVHD. Optimization of transplantation strategies could further improve these results