Background Splenectomy is a therapy for patients with treatment‐refractory autoimmune cytopenias. Antiphospholipid antibodies (aPL) can be identified in 25%–85% of these patients. In this study, we sought to identify whether the presence of aPL was associated with worse outcomes in autoimmune cytopenia's patients who had undergone splenectomy. Methods We conducted a retrospective cohort study of patients who underwent splenectomy from 2000 to 2018. We describe clinical characteristics and outcomes in patients with autoimmune cytopenia's diagnosis with positive determinations of aPL. Additionally, we performed a case–control sub‐analysis 1:1 of the cases with autoimmune cytopenia's matched control patients with negative aPL determination. Results A splenectomy was performed in 707 patients, of which we included 34 for the analysis. The median age at the time of splenectomy was 37 years (range 19–61), 53% corresponded to immune thrombocytopenia (ITP) and 47% to autoimmune hemolytic anemia (AIHA). Compared with controls (n = 34), patients had more treatment lines in addition to steroids (p = .02). There were no differences in complete response rate, 65% in cases and 80% in controls (p = .17). However, there was numerically a higher incidence of early infections (21% of cases vs. 3% controls, p = .05). During the entire follow‐up, 15% of aPL patients compared with 9% of control patients had a thrombotic event (p = .70). Discussion Splenectomy for treatment‐refractory autoimmune cytopenia's patients with persistent aPL is an effective treatment despite some safety concerns related to early infections. These results suggest that the presence of aPL should not impact the decision to undergo splenectomy.
Multiple myeloma (MM) is a common hematologic malignancy. Multi-agent chemotherapy and antimyeloma immunomodulatory drugs increase the incidence of arterial and venous thrombosis. We present a moyamoya patient with MM who had a stroke shortly after induction chemotherapy.We present the case of an adult female patient who arrived at the ER due to automatism seizures, dysarthria, and left hemiparesis. The patient had a medical history of MM and underwent six cycles of induction chemotherapy (cyclophosphamide, dexamethasone, thalidomide, and bortezomib). MRI of the brain showed bilateral watershed ischemic strokes. Angiogram showed occlusion at the supraclinoid segment of both internal carotid arteries consistent with moyamoya. The patient was discharged with full-dose anticoagulation, levetiracetam, and physical therapy. At three years of follow-up, the patient has no recurrent cerebrovascular disease.MM patients treated with thalidomide/lenalidomide in combination with high-dose dexamethasone, doxorubicin, or multiagent chemotherapy should be on anticoagulation for venous thromboembolism (VTE) prophylaxis. There are no clear recommendations for arterial thrombosis prevention. Moyamoya is a vasculopathy characterized by progressive intracranial artery stenosis with a high risk of ischemic stroke, ischemia recurrence, and intracerebral hemorrhage. Despite the risk of intracerebral hemorrhage, we decided on anticoagulation due to the high risk of thrombosis due to MM, multi-agent chemotherapy, and moyamoya.
Introduction Extramedullary myeloma (EMM) is the synchronous presence of clonal plasma cells (CPC) in both bone marrow and distant sites. Its frequency is reported among 6-20% of multiple myeloma (MM) patients and can manifest as osseous plasmacytoma (OP) or non-osseous plasmacytoma (NOP). Previous reports outline a more aggressive clinical behavior in patients presenting with EMM and in Latin American population. However, there is scarce data regarding EMM in Latin American patients. The aim of this study was to describe the clinical characteristics and impact of EMM on survival outcomes in all patients diagnosed and followed at our institution. Methods Retrospective single-center study that included >18-year-old MM patients, as defined by the IMWG criteria. EMM was defined as the presence of CPC in any osseous or non-osseous tissue outside of the bone marrow or as the demonstration of a synchronous tumor at a non-accessible site by imaging methods. Results A total of 199 patients were included. Demographic and clinical characteristics are shown in Figure 1. Sixty-three patients had EMM (31.8%), of which 45 cases (71.4%) presented at diagnosis and 18 (28.6%) at progression. Forty-eight cases (76.2%) were diagnosed by biopsy and 15 (23.8%) by imaging studies. Fifty-four percent (n=34) of the cases presented as OP and 46% (n=29) as NOP. Most-frequently involved sites included the spine and chest wall in OP and abdomen and thorax in NOP (Figure 2). EMM was associated with lower comorbidity rates and higher incidence of renal failure. Mean progression-free survival (PFS) and overall survival (OS) were 22.9 and 43.7 months, respectively. EMM at any time of disease course resulted on lower PFS and OS (15.7 months vs. 26.2 months, p=0.04; 32.1 months vs. 65.1 months, p=0.03). The presence of NOP was related with lower OS (28.4 months vs. 59.2 months, p<0.01). A higher ECOG (HR 1.62; 95%CI 1.04-2.53; p=0.034), less profound responses to treatment (HR 0.30; 95%CI 0.21-0.40; p<0.001), and the presence of EMM (HR 1.53; 95%CI 1.04-2.25; p=0.032) were associated with worse PFS; while both high ISS (HR 1.54; 95%CI 1.0-2.2; p=0.018) and less profound responses (HR 0.52; 95%CI 0.37-0.73; p<0.001) resulted in worse OS in multivariate analysis. Other factors affecting PFS and OS are shown in Figure 3. Conclusion We observed higher rates of EMM in our cohort when compared with the reported literature. The presence of EMM was associated with poor prognosis as evidenced by lower PFS and OS. When comparing OP vs. NOP EMM, NOP conferred an increased risk of early mortality. Results suggest that more aggressive disease biology, higher tumor burden, and/or delayed referral for diagnosis and therapy in our population of MM patients contribute to these unfavorable outcomes. It is imperative that our knowledge on high-risk MM groups, including EMM, in minorities is expanded. Latin American countries are urged to work together in collaborative research to confirm these results in order to properly identify patients who may benefit from more intensive treatment that might result in the improvement of long-term outcomes. Disclosures Martinez-Banos: Celgene, Amgen: Other: Advisory Board.
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