Cutaneous localization of plasmablastic multiple myeloma with heterotopic expression of CD3 and CD4: Skin involvement revealing systemic disease

2019 ◽  
Vol 46 (8) ◽  
pp. 619-622 ◽  
Author(s):  
Silvia Varricchio ◽  
Francesca Pagliuca ◽  
Antonio Travaglino ◽  
Lucia Gallo ◽  
Maria R. Villa ◽  
...  
Author(s):  
Selin Kesim ◽  
Kevser Oksuzoglu ◽  
Salih Ozguven ◽  
Tunc Ones ◽  
Leyla Cinel ◽  
...  

2018 ◽  
Vol 6 ◽  
pp. 2050313X1880399
Author(s):  
Seung Hwi Kwon ◽  
Kyung muk Jeong ◽  
Yoo Sang Baek ◽  
Jiehyun Jeon

Sarcoidosis is a multisystem inflammatory disease of unknown aetiology. Skin involvement has been reported in 12%–27% of patients with systemic disease, and scar sarcoidosis is a form of sarcoidosis developing in previous cutaneous scar areas. Scars due to all kinds of trauma, including surgery, vaccines, cosmetic tattoos, and herpes zoster infection, have been reported to be associated with sarcoidosis. Upper eyelid blepharoplasty is a mainstay of aesthetic procedure and of surgical rejuvenation of the orbital region. There have been relatively few reported scar sarcoidosis on blepharoplasty scar, considering many blepharoplasty procedures done for the last century. We report a case of 47-year-old woman presented with abruptly forming bilateral scar sarcoidosis on upper eyelid linear scars of 20 years of duration.


2018 ◽  
Vol 11 (1) ◽  
pp. e225923 ◽  
Author(s):  
Michael Uncle Williams ◽  
Caroline E Murphy ◽  
Rosco Steven Gore ◽  
Emilio Fentanes

A 58-year-old man presented with a chief complaint of tongue indentations and discomfort. Otolaryngology treated him for oral thrush with counselling to avoid tongue biting. In addition, the patient reported dyspnoea described as a decrease in tolerance of his physical activities. Due to continued increase in tongue size and worsening dyspnoea, he underwent a tissue biopsy with findings consistent with amyloidosis. Further evaluation with a bone marrow biopsy revealed underlying multiple myeloma. Echocardiography revealed abnormal ventricular wall thickness, with a reduced left ventricular chamber size, dilated atria and Doppler findings with restrictive filling patterns indicative of cardiac amyloidosis. The patient was initiated on chemotherapy for his multiple myeloma and supportive therapy for his cardiac amyloidosis. Light-chain amyloidosis (AL) is a systemic disease characterised by irreversible deposition of amyloid in tissues throughout the body; when there is cardiac involvement, it can result in heart failure with a poor prognosis. Early diagnosis of cardiac amyloidosis can lead to prolonged survival.


Blood ◽  
2021 ◽  
Vol 138 (Supplement 1) ◽  
pp. 4085-4085
Author(s):  
Megan B Sears-Smith ◽  
Lillian Charboneau ◽  
Renju Raj ◽  
R. Eric Heidel

Abstract Introduction: Autologous stem cell transplant (ASCT) is considered standard of care in young and fit patients with newly diagnosed multiple myeloma. ASCT has shown to improve depth of response, progression free survival and overall survival compared to systemic therapy alone in myeloma patients (Harousseau et al. New England Journal of Medicine). Proximity to a stem cell transplant center may influence the utilization of this therapeutic option in transplant eligible multiple myeloma patients. Our cancer center did not have a stem cell transplant program in the 100-mile driving radius. The goal of this study was to assess the referral patterns and utilization of ASCT in newly diagnosed, young (age <65 years) multiple myeloma patients in a setting where patients are lacking proximity to a transplant center. Methods: The study was an IRB-approved retrospective cohort study. Patients between 18 and 65 years of age at the time of diagnosis who were diagnosed with multiple myeloma between January 1, 2014, and December 31, 2020, were included. Data including age at diagnosis, sex, race, zip code, treatment regimen, clinical data-including referral to a transplant center, stem cell collection and transplant-were collected and analyzed. Staging was calculated using lab values at the time of diagnosis or within 2 weeks of starting treatment. Date of diagnosis was defined as the date of bone marrow biopsy confirming systemic disease. All frequency and descriptive analyses were performed using SPSS Version 26 (Armonk, NY: IBM Corp.) Results: There were n = 62 patients that met the study inclusion criteria. Patients were mainly white (86%) and male (58%) with an average age at diagnosis of 55.9 (SD = 6.83) years. All patients (n = 62, 100%) lived at zip codes that were more than 100 miles from the closest transplant center. ISS staging showed 37% (n = 23, 95% CI 25% - 50%), 29% (n = 18, 95% CI 18% - 42%), and 18% (n = 11, 95% CI 9% - 30%) to have stage I, II, and III disease respectively. Twelve patients (n = 12, 19.4%, 95% CI 10.4% - 31.4%) had insufficient data for staging. The most common first line regimens were bortezomib, lenalidomide, and dexamethasone (n = 39, 62.9%, 95% CI 49.7% - 74.8%) and bortezomib, cyclophosphamide, and dexamethasone (n = 13, 21%, 95% CI 11.7% - 33.2%). Most patients (n = 48, 77.4%, 95% CI 65% - 87.1%) achieved a very good partial response or better. Eight (n = 8, 13%, 95% CI 5.7% - 23.9%) patients had refractory disease to first line therapy. Forty-six (n = 46, 74%, 95% CI 62% - 85%) patients were referred for HSCT evaluation, n = 16 (26%, 95% CI 15.5% - 38.5%) patients were not. Of the forty-six (n = 46) patients that were referred, n = 44 (96%, 95% CI 85% - 99.5%) patients had a clinical consultation with the transplant team. Of the entire cohort, n = 36 (58%, 95% CI 44.9% - 70.5%) patients underwent stem cell collection and n = 34 (55%, 95% CI 42% - 68%) patients underwent an ASCT after induction therapy. Conclusions: Our study found that more than one third of young patients with newly diagnosed multiple myeloma did not undergo stem cell collection or stem cell transplant. Lack of geographic access to a transplant center may be a contributing factor to the under utilization of this highly effective therapeutic strategy. Further investigation into interventions to improve ASCT referral and completion rates is imperative for improving outcomes for patients in such geographic locations. Disclosures Raj: Amgen: Membership on an entity's Board of Directors or advisory committees; Jazz pharmaceuticals: Speakers Bureau; Glaxo-Smith Kline: Speakers Bureau.


JMS SKIMS ◽  
2012 ◽  
Vol 15 (1) ◽  
pp. 74-75
Author(s):  
Mushtaq Ahmad ◽  
Farhana Siraj ◽  
Mubashir Ahmad ◽  
Shabir Ahmad

The cutaneous mucinosis are a heterogenous group of disorders in which mucin accumulates in the skin. A 66 years old man presented with asymptomatic flesh coloured papules on the extremities without any truncal involvement of 3 months duration. Histopathological examination of a lesional biopsy revealed unremarkable epidermis with dermis showing pallor and loosening of collagen bundles in mucin like deposition suggestive of cutaneous mucinosis. Patient was put on steroids and hydroxycholoroquinine. Three months later he presented with encephalopathy. Investigation revealed hypercalcaemia, reversal of albumin globulin ratio, azotemia. X-ray skull revealed lytic lesions. Bone marrow aspiration and bone marrow biopsy was suggestive of multiple myeloma. This is an unknown presentation, as localized cutaneous mucinosis is not associated with systemic disease. Patient was started on chemotherapy for multiple myeloma. JMS 2012;15(1):74-75.


Author(s):  
Bernadette Lynch ◽  
Aine Burns

Scleroderma is tightness, thickening, and non-pitting induration of skin. Two forms of the skin disease are described. Limited cutaneous systemic sclerosis (lcSSc) which occurs distal to the wrists (or ankles) and/or over the face and neck, often associated with longstanding Raynaud’s phenomenon, and diffuse cutaneous systemic sclerosis (dcSSc) where truncal as well as acral skin involvement occurs as well as tendon friction rubs. In this latter condition the onset of the skin changes occurs within 1 year of onset of Raynaud’s phenomenon; however, the skin involvement may precede onset of vascular symptoms.The skin manifestations are the outward manifestation of a systemic disease, systemic sclerosis. Lung, heart, and gut involvement are frequent. Scleroderma renal crisis, usually presenting as accelerated hypertension and acute kidney injury, is one of the most severe complications of this disease. Autoantibodies against RNA polymerase are associated with scleroderma renal crisis. It occurs in 12% of dcSSc and 2% of lcSSc patients (men and women) and carries a high morbidity and mortality although careful supportive care and blood pressure management using angiotensin converting enzyme inhibitors (ACEI) or angiotensin-II receptor blockers have improved short-term outcomes. In general, beta blockers should be avoided in the early management.Approximately two-thirds of patients require dialysis, of these many recover enough function to come off dialysis. Higher blood pressure and younger age at presentation have a better prognosis. ACEIs should be continued even after dialysis is established as the latter increases the chance of late recovery. Average time to coming off dialysis is 11 months but recovery is uncommon after 24 months. After a crisis renal function continues to improve for several years.


2010 ◽  
Vol 17 (5) ◽  
Author(s):  
S. K.A. Nguyen ◽  
A. Dagnault

Author(s):  
B Guvenc ◽  
A Canataroglu ◽  
Y Gumurdulu ◽  
D Gumurdulu ◽  
S Paydas

2006 ◽  
Vol 104 (4) ◽  
pp. 608-610 ◽  
Author(s):  
Claire Haegelen ◽  
Laurent Riffaud ◽  
Marc Bernard ◽  
Beatrice Carsin-Nicol ◽  
Xavier Morandi

✓The authors describe the case of a 72-year-old woman with dural plasmacytoma revealing an immunoglobulin (Ig) G-kappa multiple myeloma (MM). She presented with headaches and left hemiparesis. Magnetic resonance imaging demonstrated a right frontal extraaxial lesion arising from the dura mater, and biological studies revealed hypercalcemia, hyperproteinemia, and a serum gamma globulin peak. A diagnosis of IgG-kappa MM was based on microscopic examination and immunohistochemical analysis of the dural plasmacytoma as well as on signs of systemic myeloma after surgery. The patient died 3 years after the first symptoms of MM despite systemic chemotherapy and no recurrence of the dural plasmacytoma. Myelomatous involvement of the dura mater is a rare occurrence given that only three cases have been reported to date. Nevertheless, this pathological entity should be differentiated from solitary dural plasmacytoma (SDP) because the prognosis is radically different. Progression seems to be correlated with systemic disease in contrast to the long-term survival associated with SDP. Careful systemic evaluation should be made in such a presentation to rule out MM, which would require different management and has a different prognosis.


1997 ◽  
Vol 2 (2) ◽  
pp. 100-103 ◽  
Author(s):  
J. Mark Jackson ◽  
Jeffrey P. Collen

Background: Pyoderma gangrenosum (PG) is an uncommon condition that is associated with a systemic disease in 50% of patients. The condition may be associated with a monoclonal gammopathy, usually of the IgA type. It is rare for PG to be associated with multiple myeloma. Observations: We report the case of an 83-year-old man with PG associated with an IgA myeloma. The myeloma was discovered after the diagnosis of PG had been made. This is the 22nd case of multiple myeloma associated with PG, and only the 14th case of PG with an IgA myeloma. Conclusions: We should be aware of the potential association of multiple myeloma with PG, and consider doing a serum protein electrophoresis in the evaluation of patients with PG.


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