SummaryChanges in spleen size postallogeneic haematopoietic stem cell transplantation (HSCT) in patients with primary myelofibrosis have been poorly characterized. We analysed 10 patients with myelofibrosis and splenomegaly following a reduced-intensity allogeneic HSCT. All patients fully engrafted donor cells including five patients with extensive splenomegaly. Extensive splenomegaly was associated with a prolonged time to neutrophil and platelet recovery. In all 10 patients, a progressive reduction of splenomegaly was documented within 12 months posttransplant and paralleled the reduction of marrow fibrosis. These findings suggest that myelofibrosis patients with extensive splenomegaly may proceed with allogeneic HSCT without prior splenectomy.
Objective. Primary renal candidiasis is rare but increasing in incidence. The purpose of this series is to provide imaging and clinical findings for diagnosing candidiasis and to discuss imaging in the management of this disease. Methods. Ten sonographic, 8 retrograde pyelographic, 2 intravenous pyelographic, 2 antegrade pyelographic, and 2 computed tomographic examinations of 5 patients (4 adult male patients and 1 16-year-old female patient) were reviewed. Results. The clinical presentation was variable. Sonography showed renal pelvic wall thickening (n = 5), echogenic debris (n = 4), and fungus balls (n = 2). Papillary necrosis (n = 4), filling defects due to debris (n = 3), and fungus balls (n = 2) were seen on retrograde pyelography. Conclusions. Untreated candidiasis may progress to fungus ball or abscess formation. Sonography is commonly used as the initial imaging procedure. Retrograde and antegrade pyelography are used for biopsy, diagnosis, and treatment. Awareness of this condition and knowledgeable imaging evaluation can help detect and define the site, infection severity, and subsequent therapy.
Allogeneic stem cell transplantation has been shown to restore normal hematopoiesis in patients with intermediate or high risk primary myelofibrosis (PMF) or myelofibrosis preceeded by polycythemia vera or essential thrombocythemia. However, in patients with PMF and extensive splenomegaly it is not clear whether transplant is associated with an unacceptable risk or if splenectomy should be performed prior to transplant. In this study, ten consecutive patients with myelofibrosis who were not splenectomized received an allogeneic hematopoietic stem cell transplantation (HSCT) from related or unrelated donors and were periodically monitored by ultrasound or CT scan for 12 months after transplant to assess the kinetics of the reduction of splenomegaly. These findings were correlated with the time to resolution of marrow fibrosis, time to engraftment and clinical outcome. Over a 12 month period a progressive reduction in spleen size was observed in all the patients and paralleled the reduction in marrow fibrosis (Figure 1). Of 10 patients, 5 with a splenic longitudinal diameter >30 cm showed a more prolonged time to engraftment ANC> 0.5 × 109/L (d 19±5 vs 13±2, p=0.05) and platelet > 20 × 109/L (d 75±104 vs 11±2, p=0.06). However, of the 5 patients with more extensive splenomegaly only 2 experienced delayed engraftment of platelets (d 77 and 256, respectively). Full donor chimerism was observed in all patients within 60 days after transplantation regardless of the size of the spleen. At a median follow-up of 54 months (range: 2–80) all the patients are alive but 1 who died of a TTP like syndrome 2 months after transplant and had a small spleen at the time of transplant. A clinical CR has been documented in 7 patients and 2 have achieved clinical improvement as assessed by IWG response criteria. These findings suggest that allogeneic HSCT with RIC regimen can be safely performed in patients with extensive splenomegaly. Although in these patients the time to engraftment may be prolonged, the significant risk of morbidity and mortality associated with splenectomy may be avoided.
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