Spontaneous spleen rupture: an unusual presentation of extramedullary multiple myeloma

Abreu, Maria Eduarda Bini de; Farias, João Samuel de Holanda; Lima, Renata Bernardini de; Campos, Giulia

doi:10.1016/j.htct.2019.10.004

Cited by 1 publication

(2 citation statements)

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“…At present, treatment options include splenectomy, chemotherapy and autologous stem cell transplantation according to reported The clinical features of the newly diagnosed MM with spleen involvement. [4][5][6][7][8][9][10][11] Author Perry-Thornton et al [4] Sherwood et al [5] Ho et al [6] Magnoli et al [7] Peng et al [8] Abreu et al [9] Mello et al [10] Liu et al [11] Present experiences. Similar to a study of relapsed/refractory EMM, the present case appears to benefit more from chemotherapy than new drug therapy.…”

Section: Discussionmentioning

confidence: 99%

“…[3] In particular, spleen involvement in MM at the time of initial diagnosis is extremely rare, and to our knowledge, as few as 8 cases have been published in the literature to date. [4–11] Most of these cases were reported as splenic rupture secondary to MM, with overall survival <1 year or even several days. [5–7] Herein, we describe an unusual case of spleen infiltration in a newly diagnosed MM patient whose initial manifestation was megalosplenia, an atypical manifestation of MM.…”

Section: Introductionmentioning

confidence: 99%

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Megalosplenia as an initial manifestation of multiple myeloma with a novel CYLD gene mutation: A case report and literature review

Zhang,

Zhang

2024

Medicine

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Introduction: Megalosplenia in newly diagnosed multiple myeloma (MM) is extremely rare, posing diagnostic and therapeutic challenges due to its unusual location and clinical manifestations and lack of optimal therapeutic strategies. Case presentation: A 65-year-old female who was previously healthy presented with a history of ecchymosis on her right leg accompanied by progressive fatigue for 2 weeks. She was admitted to our center in July 2019 due to thrombocytopenia. The patient presented with megalosplenia, anemia, monoclonal protein (λ-light chain type) in the serum and urine, and 45.6% malignant plasma cells in the bone marrow. Splenectomy was performed due to persistent splenomegaly after 3 cycles of the bortezomib plus dexamethasone regimen, and immunohistochemistry results indicated λ-plasmacytoma of the spleen. The same cytogenetic and molecular abnormalities, including t(14;16), 14q32 amplification, 16q32 amplification, 20q12 amplification, and a novel CYLD gene mutation, were identified using fluorescence in situ hybridization and next-generation sequencing in both bone marrow and spleen samples. Therefore, a diagnosis of MM (λ-light chain type, DS III, ISS III, R-ISS III, high-risk) with spleen infiltration was proposed. The patient did not achieve remission after induction treatment with bortezomib plus lenalidomide and dexamethasone or salvage therapy with daratumumab plus ixazomib and dexamethasone. However, she ultimately did achieve very good partial remission with a regimen of bendamustine plus lenalidomide and dexamethasone. Unfortunately, she died of pneumonia associated with chemotherapy. Conclusion: To our knowledge, only 8 cases of spleen plasmacytoma at MM diagnosis have been described previously. Extramedullary myeloma patients with spleen involvement at diagnosis are younger and that the condition is usually accompanied by splenic rupture with aggressive clinical features and poor prognosis. Further studies are needed to explore pathogenesis and effective therapies to prolong the survival of such patients.

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