Clinical features and prognosis of multiple myeloma and orbital extramedullary disease:Seven cases report and review of literature

BACKGROUND Multiple myeloma(MM)complicated with extramedullary disease(EMD)has a poor prognosis and is a limiting factor in the treatment of MM,and no standard treatment is recommended in international guidelines.Few studies have reported MM with periorbital EMD.CASE SUMMARY In this paper,the clinical characteristics and survival of seven patients with multiple myeloma and orbital are described and analyzed.The common ocular symptoms were blurred vision,proptosis and/or eye movement disorders,IgG type MM may be a risk factor for orbital involvement.Of them,six patients were treated with bortezomib-based regimens.The median overall survival(OS)and progression free survival for the entire cohort were 48 and 33 mo,respectively,which was much worse than the OS reported for MM patients without orbital EMD.CONCLUSION Orbital MM may have significantly shortened survival for the entire cohort,so multidisciplinary collaboration is emphasized and recommended in the diagnosis and treatment of these difficult cases.

Novel agents for multiple myeloma with extramedullary disease

Multiple myeloma(MM)is the second most common hematological malignancy.The introduction of proteasome inhibitors(PIs)and immunomodulatory drugs(IMiDs)greatly improved the outcomes for these patients.Extramedullary disease(EMD),an aggressive manifestation of MM,remains a significant treatment challenge.Recently,active clinical development of remarkable novel agents for MM with new mechanisms of action has begun.These agents show promising anti-MM effects and provide the opportunity to extend the survival of patients.They include next-generation PIs and IMiDs;monoclonal antibodies;chimeric antigen receptor T cells;antibody-drug conjugates;bispecific T cell engager antibodies;and small molecular inhibitors.However,few clinical trials have focused on patients with EMD,and the effectiveness of these agents has not been well evaluated and compared in these patients.In this review,we summarize the efficacy of these novel agents in MM patients with EMD.

Myeloid sarcoma of the colon as initial presentation in acute promyelocytic leukemia:A case report and review of the literature

BACKGROUND Myeloid sarcoma(MS)rarely occurs in acute promyelocytic leukemia(APL)at onset,but it can develop in relapse cases,especially after APL treated with alltrans retinoic acid(ATRA).Therefore little is known about the clinical features and suitable treatment for APL related MS due to the rarity of the disease,although this may be different from the treatment and prognosis of MS in the relapse stage.To our best knowledge,this is the second case report of APL initial presentation as colon MS.CASE SUMMARY A 77-year-old woman complained of intermittent right lower abdominal pain,black stool,and difficult defecation for 2 mo.Physical examination showed diffuse tenderness during deep palpation and an anemic appearance.Laboratory findings showed positivity for fecal occult blood testing;white blood cell count:3.84×109/L;hemoglobin:105 g/L;platelet count:174×109/L;and negativity for tumor markers.Abdominal enhanced computed tomography showed a space occupying lesion in the colon(1.9 cm).Fibrocolonoscopy revealed a polypoid and ulcerated mass measuring 2.5 cm.The tumor was removed.To our surprise,MS was confirmed by immunohistochemistry.PML/RARαfusion gene was detected in colon specimens by fluorescent in situ hybridization and real-time reverse transcription polymerase chain reaction,which was consistent with the bone marrow.She was diagnosed as having APL related MS.A smooth and unobstructed intestinal wall was found by fibrocolonoscopy,and continuous molecular remission was confirmed in both the bone marrow and colon after four courses of ATRA+arsenic trioxide(ATO).ATRA+ATO showed a favorable therapeutic response for both APL and MS.CONCLUSION Early use of ATRA can benefit APL patients,regardless of whether MS is the first or recurrent manifestation.