The sarcoidosis-lymphoma syndrome

Brincker, H

doi:10.1038/bjc.1986.199

Cited by 267 publications

(166 citation statements)

References 22 publications

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“…Indeed, the existence of a 'sarcoid-lymphoma' syndrome has been proposed which is characterised by onset of lymphoma after the development of sarcoidosis. 5 In general, these features hold true for the reported associations between plasma cell dyscrasias and sarcoidosis. 8 The pathogenesis of DM or SLRs associated with malignancy is not known, but presumably involves a host immune response to tumour antigens.…”

Section: Discussionmentioning

confidence: 65%

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Dermatomyositis and sarcoid-like reaction associated with multiple myeloma treated effectively by high-dose chemotherapy and autologous peripheral blood stem cell transplantation

Chakraverty

Rabin

Peggs

et al. 2001

Bone Marrow Transplant

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Summary:We report the case of a 46-year-old male who developed dermatomyositis and a sarcoid-like reaction in association with testicular relapse of multiple myeloma. The myositis progressed despite chemotherapy directed at the underlying malignant disorder and immunosuppressive treatment. There was, however, a dramatic and sustained response to high-dose chemotherapy and autologous peripheral blood stem cell transplantation which resulted in resolution of the myopathy and partial resolution of the sarcoid-like reaction. This case report highlights the potential of autologous stem cell transplantation as treatment for para-neoplastic disorders associated with haematological malignancies. Bone Marrow Transplantation (2001) 27, 1215-1217. Keywords: multiple myeloma; autologous PBSCT; dermatomyositis; sarcoidosis The presenting clinical features of multiple myeloma are protean and reflect the direct effects of clonal plasma cell proliferation (eg bone marrow failure), the activity of monoclonal antibody (eg amyloid deposition), or the consequence of a complex inter-relationship between the abnormal clone and the host bone marrow micro-environment (eg bone disease). The pathogenesis of the various 'paraneoplastic' disorders which occur in association with myeloma, such as axonal or demyelinating neuropathy, is less certain although antibodies with specificity for proteins expressed by affected tissues, amyloid deposition or changes in cytokine production patterns have all been proposed as potential mechanisms. The optimal treatment for this group of disorders has not been defined and their impact upon prognosis is not known. We report for the first time the effectiveness of high-dose chemotherapy and peripheral blood stem cell transplantation (PBSCT) as treatment for a para-neoplastic disorder associated with multiple myeloma. Case reportA 46-year-old man presented with a 3 month history of general malaise and 2 week history of vomiting in March 1998. There was no significant past medical history. On examination he appeared unwell and short of breath at rest. He had a raised venous pressure, peripheral oedema and bilateral inspiratory crepitations on chest examination. FBC at presentation was: Hb 8.2 g/dl, white cell count 5.2 × 10 9 /l and platelets 163 ϫ 10 9 /l. Renal function tests confirmed acute renal failure with a creatinine of 844 umol/l (normal range 62-130) and urea 46.4 mmol/l (normal range 2.8-7.6). Corrected serum calcium was 3.43 mmol/l. Chest Xray showed changes consistent with pulmonary oedema. Serum electrophoresis confirmed an IgG lambda paraprotein of 70 g/l. No urinary Bence-Jones protein was detected. Skeletal survey revealed no lytic lesions. Bone marrow trephine was diffusely fibrotic with deposition of collagen (grade 4) and heavy infiltration by neoplastic plasma cells (CD138 positive, lambda light chain restriction). Most plasma cells had the features of plasmblasts. Renal biopsy showed a cast nephropathy.The diagnosis was of IgG lambda myeloma complicated by hypercalcaemia and acute rena...

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Section: Discussionmentioning

confidence: 65%

“…Although individual associations between lymphoproliferative disorders (LPDs) and both DM 4 or SLRs 5 have been reported previously, to our knowledge there are no descriptions of this triad. DM is associated with a five-to seven-fold increase in the incidence of malignancy 4 includ-ing in some, but not all studies, associations with LPDs.…”

Section: Discussionmentioning

confidence: 85%

Dermatomyositis and sarcoid-like reaction associated with multiple myeloma treated effectively by high-dose chemotherapy and autologous peripheral blood stem cell transplantation

Chakraverty

Rabin

Peggs

et al. 2001

Bone Marrow Transplant

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“…T cells. In addition, this chronic stimulation may result in prolonged B-cell and plasma cell halflives that may increase the risk of genetic mutations, leading to the formation of a malignancy, such as multiple myeloma [18].…”

Section: Discussionmentioning

confidence: 99%

Atypical sarcoidosis diagnosed by bone marrow biopsy during renal workup for possible multiple myeloma

et al. 2012

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Sarcoidosis is a multi-organ disease of unknown etiology characterized by non-caseating granulomas. Here we report the case of a 78-year-old white male with a past medical history of diabetes mellitus, hypertension, and chronic kidney disease stage III with a baseline serum creatinine of 2.5 mg/dl. The patient had a prior admission history for acute kidney injury (AKI) attributed to dehydration and medication-induced nephro-toxicities. He presented to the renal clinic for follow-up with acute worsening of chronic kidney failure with a serum creatinine level of 3.5 mg/dl. Examination revealed that he was anemic and mildly hypercalcemic with suppressed parathyroid hormone and had proteinuria of 1.3 g per day. The computed tomography scan of the abdomen revealed right renal pelvic non-obstructing calculi. Serum protein electrophoresis revealed gammopathy with two distinct monoclonal peaks consisting of immunoglobulin G (IgG) kappa and IgG lambda, respectively. The kappa/lambda ratio was within normal limits, and urine protein electrophoresis showed no evidence of a monoclonal peak or Bence Jones proteins. Further workup for multiple myeloma, including bone marrow (BM) biopsy, revealed polyclonal plasma cells and B cells with no clonality. No morphological and immunephenotypic evidence of plasma cell dyscrasia was found, but BM biopsy did show numerous non-caseating granulomas consistent with sarcoidosis. Skin biopsy from non-scaly 6-mm skin colored papule also showed non-caseating granulomas. The patient had elevated angiotensinconverting enzyme levels (165 ug/l) and an erythrocyte sedimentation rate of 27 mm/h. Kidney biopsy did not show granulomas. The hypercalcemia, proteinuria, and AKI responded well after 2 weeks of 60 mg oral prednisone daily.

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“…Second, Brincker [7] reviewed 46 cases of lymphoma occurring in patients with sarcoidosis. He reported only 2 cases in which lymphoma preceded sarcoidosis with an additional 2 cases in a subsequent study [9].…”

Section: Introductionmentioning

confidence: 99%

“…Since then, additional descriptions of this possible connection have been published but without providing a better understanding of this relationship [1]. The sarcoidosis-lymphoma syndrome descried by Brincker [7] [8] was established on the basis of 2 different studies. In a Danish registry of patients with respiratory sarcoidosis, lymphoma occurred 11.5 times more frequently than expected in the general population [8].…”

Section: Introductionmentioning

confidence: 99%

Sarcoidosis-Lymphoma Syndrome: A Spectrum of One Disease

Sharquie¹,

Al-Hayani²,

Abdulwahhab³

et al. 2015

JCDSA

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Lymphoma might occur in association with sarcoidosis or sarcoidosis might be combined with lymphoma, in so called ("sarcoidosis-lymphoma syndrome"). This syndrome is well reported in medical literature. The question, which one starts before is difficult to answer, as in some cases sarcoidosis starts first then is followed by lymphoma but in other cases during the course of lymphoma sarcoid reaction might be seen. In the present report, we describe a case of 60 years male patient that is presented with a rash with classical sarcoid pathology that overtime changes into typical lymphoma ended with death.

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The sarcoidosis-lymphoma syndrome

Cited by 267 publications

References 22 publications

Dermatomyositis and sarcoid-like reaction associated with multiple myeloma treated effectively by high-dose chemotherapy and autologous peripheral blood stem cell transplantation

Dermatomyositis and sarcoid-like reaction associated with multiple myeloma treated effectively by high-dose chemotherapy and autologous peripheral blood stem cell transplantation

Atypical sarcoidosis diagnosed by bone marrow biopsy during renal workup for possible multiple myeloma

Sarcoidosis-Lymphoma Syndrome: A Spectrum of One Disease

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