Spontaneous splenic rupture due to isolated splenic peliosis in a case of multiple myeloma

Abstract

A 59-year-old male was diagnosed with asymptomatic IgG (lambda) multiple myeloma. He was kept under observation and 6 years later developed renal impairment; therapeutic options were discussed. Shortly afterwards he suffered a large perisplenic bleed (left, computed tomography scan). He required emergency splenectomy and transfusion of 17 units of blood. Histopathological examination revealed peliosis (right) with occasional plasma cells. These were polyclonal by kappa and lambda light chain in situ hybridization. The patient made an uneventful recovery and was subsequently commenced on cyclophosphomide, thalidomide and dexamethasone, to which he responded well.Peliosis (blue/black in Greek), is a condition characterised by blood-filled cavities dispersed throughout the parenchyma of solid organs. Splenic peliosis has been reported in association with haematological disorders including aplastic anaemia, hereditary spherocytosis, myelofibrosis, myeloma and Hodgkin lymphoma. It has been suggested that males are more at risk and an association with the use of androgenic steroids, anti-cancer drugs, the acquired immunodeficiency syndrome and tuberculosis has been reported.Central to the pathogenesis is the perifollicular region of the spleen, with injury to the sinusoidal wall. Light chain deposits along the annular fibres circling the red pulp sinuses form an obstacle to blood cell migration through the sinus walls, resulting in the accumulation and death of cells within the cords. Macrophages and reticuloendothelial cells neighbour the white pulp causing diminished blood flow and preferential deposition of the particulate antigens and toxins.Splenic rupture requires urgent operative intervention. Once the presence of peliosis has been demonstrated investigation to detect its presence in other organs and to establish a possible cause is indicated. Physicians should be mindful of this rare but potentially life threatening condition in multiple myeloma.

Spontaneous splenic rupture due to Peliosis and the association with malignancy: A case series and literature review

“…Patients with splenic peliosis are commonly asymptomatic, and splenic peliosis may present as an incidental finding on imaging, at autopsy, or with spontaneous rupture after minimal or no trauma [ [1] , [2] , [3] , [4] , [5] , [6] , [7] , [8] , [9] , [10] , [11] , [12] , [13] , [14] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , 34 , [39] , [40] , [41] , [42] , [43] ]. Peliosis discovered on imaging should immediately prompt further evaluation.…”

“…However, the condition may also be recognized as an incidental finding on computed tomography (CT) scans or at autopsy [ [2] , [3] , [4] , [5] , 8 , [17] , [18] , [19] ]. Numerous associations with medications and other conditions have been described in the literature, emphasizing the limited understanding of this condition [ [2] , [3] , [4] , [5] , [15] , [16] , [17] , [18] , [19] , [20] , [21] , [22] , [23] , [24] , [25] ]. The aim of this retrospective, single-site, case series is to highlight the urgency required for the care of patients who present with spontaneous splenic rupture due to splenic peliosis, and to draw attention to the association with hematologic malignancy.…”

An Autopsy Case of Spontaneous Splenic Rupture Due to Isolated Splenic Peliosis and Review of the Literature

“…These include Hepatitis C virus infection, liver cirrhosis, tuberculosis, AIDS, post-transplant immunodeficiency, intravenous drug abuse, chronic alcoholism, androgenic steroid therapy, corticosteroid therapy, oral contraceptives, advanced malignancies including hematologic malignancies, and hemodialysis. To date, spontaneous rupture of the spleen due to peliosis has been previously described few times in the literature (7)(8)(9)(10)(11)(12)(13)(14)(15)(16). Peliosis of the spleen has characteristic gross appearance of cystic blood-filled spaces with well-defined margins, commonly involving the red pulp, distributed in a sporadic, clustered, or disseminated pattern.…”

Spontaneous splenic rupture during induction therapy in acute myeloid leukemia: An unusual case.

“…Peliosis is an extremely uncommon disorder of unknown etiology, and only a few cases are described in hematological settings. [4] , [5] , [6] Many reports have associated clinical factors including malignant diseases, chronic infections, and ingestion of certain drugs such as anabolic steroids and long-term erythropoietin. [7 , 8] Although uncommon, some reports suggest that males, ages > 20 years, presence of splenomegaly, and use of cytoreductive therapy might be associated with increased risk of SSR in hematology.…”