Spontaneous rupture of the spleen in AL amyloidosis

Abstract

The frequency of splenic involvement in AL amyloidosis is not precisely known. However, splenomegaly has been reported in 4-13% of patients. We report four cases of spontaneous splenic rupture in patients with AL amyloidosis. Splenic rupture was the initial manifestation of the disease in one of these patients. The other three experienced splenic rupture during or after high-dose intravenous melphalan with autologous peripheral blood stem cell transplantation (HDM/SCT): one during stem cell mobilization with G-CSF prior to HDM/SCT and two after hematopoietic recovery following treatment. Two of the four patients had Factor X deficiency, the most common coagulation abnormality associated with AL amyloidosis. All four patients underwent splenectomy without significant postoperative complications. Splenic rupture in AL amyloidosis as a complication of aggressive treatment with HDM/SCT has not been reported previously. Am.

Transplantation without growth factor: engraftment kinetics after stem cell transplantation for primary systemic amyloidosis (AL)

“…7 Splenic rupture during stem cell mobilization in the presence of splenic amyloidosis has been reported. 8 Investigators at Boston University reported two patients in whom splenic rupture developed because of growth factor administration. Presumably, the patients had extensive amyloid deposits in the spleen, rendering the capsule rigid during the expansion of neutrophils after filgrastim therapy.…”

Splenectomy in plasma cell dyscrasias: A review of the clinical practice

“…In 2003, Oran et al [24] reported 4 cases of splenic rupture in AL amyloidosis in this journal. Three of these cases were complicated by splenic rupture during or after treatment with HDM/ASCT.…”

“…A rare phenomenon associated with high mortality, spontaneous rupture of the spleen can occur in AL amyloidosis because of splenic enlargement, increased rigidity, amyloid angiopathy, and clotting factor deficiency. Preservation of the spleen is usually not possible in such cases [23,24]. G-CSF used during high dose Melphalan chemotherapy and autologous stem cell transplantation (HDM/ASCT) has also been implicated in causing the rupture of this friable organ, possibly by continuous proliferation of the stimulated hemopoietic stem cells [24].…”

“…All the aforementioned cases underwent open splenectomies. Due to the containment of the rupture by an organized hematoma, and no major active hemorrhage perioperatively, the authors proposed to attempt laparoscopic splenectomies if they were confronted with similar patients in the future, provided the cardiac status of those patients permitted such an approach [24].…”

“…It has been reported to occur in 6.3-14% of these patients [24,[27][28][29]. Factor X deficiency has long been thought to result from adsorption or the in vivo binding of the factor to the amyloid fibrils [30,31].…”