Long Term Remission of Capillary Leak Syndrome Associated with Monoclonal Gammopathy with Progression to Multiple Myeloma After Autologous Stem Cell Transplantation: a Case Report and Review of the Literature.

BACKGROUND: Clarkson's disease is a very rare entity characterised by acute episodes of systemic oedema and severe hypotension associated with paraproteinaemia. Its classical treatment relies on methylxanthine combined with terbutaline. Although this prophylactic therapy reduces the mortality rate, relapses are frequent. Eighty percent of patients with Clarkson's disease present with monoclonal gammopathy of unknown significance (MGUS). The risk of progression to multiple myeloma is 1% per year. CASE DESCRIPTION: Here, we present a 49-year-old woman who suffered multiple such episodes requiring treatment in the intensive care unit. Treatment with terbutaline and theophylline was ineffective. She was diagnosed with multiple myeloma (MM) 8 years after the first of these acute episodes. Antimyeloma treatment with bortezomib and dexamethasone was started, followed by autologous haemopoietic transplantation, with no further acute episodes since then. CONCLUSION: Our case is, to our knowledge, unique because eradication of MM was followed by complete disappearance of acute episodes of capillary leakage. Our case report is also the first to support the use of bortezomib and dexamethasone in this setting. Furthermore, autologous peripheral blood progenitor cell transplantation consolidated the MM stringent complete remission achieving a very long progression-free survival (> 11 years) of both MM and Clarkson's disease.

Dasatinib and FLAG-IDA Is an Effective Therapy for Initial Myeloid Blast Crisis but Involves a High Risk of Opportunistic Infections.

Blast crisis (BC) continues to be the major challenge in the treatment of chronic myeloid leukemia. Best results have been observed in a few patients who were successfully transplanted after returning to chronic phase. Recent studies focus on the combination of chemotherapy with imatinib, but results remain unsatisfactory. Since dasatinib induces deeper and faster responses, a reasonable strategy might be to combine it with chemotherapy, taking into account the alterations in T-cell response induced by dasatinib. However, there are no published studies or case reports supporting the use of dasatinib as first line treatment for initial myeloid BC, and very little is known about infectious complications associated with this drug. Based on this, we present the case of a patient diagnosed with an initial nonlymphoid phenotype BC, who achieved molecular response (MR4.5) with dasatinib and FLAG-IDA, but he suffered a pulmonary aspergillosis, CMV infection, and a CMV reactivation, prior to an allogeneic hematopoietic stem cell transplantation (HSCT). In conclusion, dasatinib and FLAG-IDA is an effective therapy for initial BC. However, a warning call is needed owing to the high risk of opportunistic infections, such as CMV.