ABSTRACT
INTRODUCTION: Acquired angioedema is a rare disorder causing recurrent life-threatening angioedema, due to decreased activity of C1 esterase inhibitor. CASE REPORT: A 57-year-old man presented to our hospital with recurrent swelling of the hands, lips, tongue, scrotum and throat. Lab examination showed the presence of an IgM kappa monoclonal antibody. Additional analysis showed that in the IgM fraction autoantibody activity against C1 esterase inhibitor was present. This confirmed the diagnosis of acquired angioedema in the presence of lymphoplasmacytic lymphoma. Despite standard therapy, there was an increase in the episodes of laryngeal oedema. Therefore it was decided to perform a non-myeloablative allogeneic haematopoietic stem cell transplantation, with his HLA-identical brother as donor. The post-transplantation course was without complications. Five years following alloSCT he is in complete remission without symptoms and with increased C1 esterase inhibitor activity. DISCUSSION: In this case all other known treatment options for severe acquired angioedema failed. This is the first case describing treatment of severe acquired angioedema, caused by lymphoplasmacytic lymphoma, with an alloSCT.
Subject(s)
Angioedema/etiology , Angioedema/therapy , Hematopoietic Stem Cell Transplantation , Waldenstrom Macroglobulinemia/complications , Waldenstrom Macroglobulinemia/therapy , Hematopoietic Stem Cell Transplantation/methods , Humans , Male , Middle Aged , Transplantation, HomologousABSTRACT
We determined the argon (Ar) isotope ratio in samples of expired alveolar gas gathered during Ar washout from residual gas relative to this ratio in samples of expired alveolar gas gathered just before the beginning of this washout in 13 young, healthy human subjects at rest. These data were determined for a limited number of breaths in early washout and were used to calculate the relative difference between the alveolar ventilations of (36)Ar and (40)Ar (Delta(rel)V (A)((36)Ar,(40)Ar)). Mean Delta(rel)V (A)((36)Ar,(40)Ar) amounted to 1.6 per thousand (S.D.=1.3 per thousand). This result was then used to discuss the contribution of intrapulmonary gas mixing by diffusion to oxygen isotope fractionation of alveolar gas by respiration. On the basis of our finding for Delta(rel)V (A)((36)Ar,(40)Ar) and further theoretical considerations we arrived at the conclusion that this contribution for subjects at rest is small (about 1 per thousand) and that this contribution is negative irrespective of the level of exercise.
