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Bone marrow biopsies demonstrating non-necrotizing granulomas before steroid therapy (100× original magnification) (a) and follow-up bone marrow biopsy 5 months after diagnosis (4 months later after steroid therapy) shows partial resolution of granulomas without evidence of malignancy (200× original magnification) (b)
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Background
Bone marrow sarcoidosis is extremely rare. The association between sarcoidosis and lymphoproliferative disorders has been previously speculated, although the diagnosis of sarcoidosis often precedes any hematological derangements. Case presentationHere, we report for the first time, a case of a 57-year-old Caucasian woman with a previous...
Contexts in source publication
Context 1
... was a concern for a neoplastic process, particu- larly myeloma, given her history of MGUS. The patient underwent bone marrow biopsy at the time of discharge, which was remarkable for non-necrotizing granulomas with no findings suggestive of plasma cell dyscrasia or other neoplasms. (Fig. 1a) Two weeks later, the patient's repeat calcium was 12.7 mg/dL despite adequate fluid intake. The patient was admitted for further evaluation of hypercalcemia and bone marrow granulomas. An extensive hypercalcemia workup was unrevealing. Malig- nant and infectious etiologies of granulomatous disease in this patient were investigated. ...
Context 2
... After discontinuation of prednisone, she developed hypercalcemia again. She was then treated with a slow prednisone taper for four months with normalization of serum calcium levels. A follow-up bone marrow biopsy 5 months later (1 month after 4-month course of slow prednisone taper) showed partial resolution of granulomas without malignancies. (Fig. 1b) At her follow-up PCP visit, two years after her initial presentation, the patient was overall asymptomatic with normal serum calcium, ACE, and CBC. ...
Citations
... However, there is a dearth of case reports describing the relationship of extrapulmonary sarcoidosis with plasma cell dyscrasias in particular. 4,5 Multiple myeloma is a haematological malignancy arising from the abnormal monoclonal proliferation within the bone marrow of malignant plasma cells. 6 This case report describes a case of disseminated sarcoidosis simultaneously diagnosed with smouldering multiple myeloma as per the International Myeloma Working Group (IMWG) diagnostic criteria which later evolved into multiple myeloma. ...
... 9 Based on the population investigated, presence of hypercalcemia in sarcoidosis patients can vary between 2-63%; the reason for such wide disparities in frequency of hypercalcemia has been ascribed to the diversity in blood calcium testing in addition to the fluctuating disease course of sarcoidosis. 5,10 Conclusion A patient presenting simultaneously with sarcoidosis and smouldering MM at the time of diagnosis is extremely unusual, and transforming into MM is even rarer. Hence, dismissal as a mere coincidence should be disdained as prospective epidemiological research will largely depend on it, and a strong index of suspicion is warranted with thorough workup and very close follow-up as it bears a dismal prognosis. ...
... 10 Although there is no randomized trial to definitively compare treatment modalities of bone marrow sarcoidosis, steroids appear to be the preferred method of treatment amongst published cases. 11 When a patient progresses or does not respond to steroids, other options include various immunosuppressants such as azathioprine, methotrexate, or adalimumab. 9,12 The combination of prednisone and azathioprine was successful in the treatment of the patient discussed. ...
Patients with granulomatous disease often have widespread pulmonary and extrapulmonary disease. In the absence of this, a search of the pulmonary, renal, hepatic, ocular, and bone marrow is warranted in the setting of hypercalcemia with unexplained elevated 1,25-dihydroxyvitamin D, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP). We present a case of hypercalcemia and a decline in renal function in a patient with bone marrow sarcoidosis. A 45-year-old woman was admitted to the hospital after hypercalcemia, acute kidney injury, and pancytopenia were found on a routine outpatient lab. She was discharged after improvement with IV fluids. She had interval worsening of hypercalcemia and was readmitted within a week for pamidronate treatment. Imaging and labs were concerning for sarcoidosis, but bronchoscopy with biopsy was nondiagnostic. Eventual bone marrow biopsy confirmed evidence of granulomas. Her condition improved with prednisone over 3 months and ultimately, azathioprine. Non-parathyroid hormone-mediated hypercalcemia should be thoroughly worked up for a source to rule out malignancy and to diagnose treatable causes such as sarcoidosis. Sarcoidosis may not present in its traditional pulmonary pattern, necessitating further diagnostic measures such as a bone marrow biopsy.
... The risk of progression of MGUS to multiple myeloma is approximately 1% per year [1]. Although MGUS or multiple myeloma in association with sarcoidosis is not well elucidated, 10 such cases were previously reported [2,3]. Additionally, 33 cases with both sarcoidosis and multiple myeloma were also reported [4]. ...
... As the one side of sarcoidosis, lymphoproliferative neoplasm is known to occur concomitantly with sarcoidosis [5]. One explanation for the relationship between sarcoidosis and MGUS is that the hypergammaglobulinemia induced by the chronic inflammatory response with sarcoidosis results in prolonged plasma cell lifespan [2][3][4]. Due to the risk of progression to multiple myeloma, a careful follow-up is required. ...
... Childhood sarcoidosis is a multisystem inflammatory disorder that can involve any organ but most frequently affects lymph nodes, lungs, liver, spleen, skin, eyes, musculoskeletal system, kidney (6%), brain, heart (conduction abnormality), and deranges the calcium metabolism (3,6,19,20). However, bone marrow involvement in pediatric sarcoidosis is not known. ...
Childhood sarcoidosis is a chronic multisystemic, non-caseating granulomatous disease of unknown etiology. Early-onset disease classically presents with a triad of skin rash, uveitis, and arthritis, but bone marrow involvement is rare. We report a 9-1/2-year old Indian female child who presented with bleeding manifestation, skin rash, uveitis, and arthritis. Bone marrow biopsy showed multiple non-necrotizing granulomas comprising epithelioid cells, mature lymphocytes, and multinucleated giant cells with few eosinophils in the background, with negative staining for acid-fast bacilli or fungi. She was treated successfully with oral prednisolone. This is the first report of an early-onset childhood sarcoidosis with bone marrow involvement from India.
... To the best of our knowledge, there are no randomized controlled trials comparing different therapeutic strategies for BMS [50]. Prednisone remains the mainstay of treatment. ...
Sarcoidosis is a granulomatous disease with various extrapulmonary manifestations. We describe a 51-year-old African American woman with a history of cutaneous sarcoidosis admitted with bicytopenia. Suspicion for systemic sarcoidosis was established after contrast-enhanced computerized tomography of the chest, abdomen and pelvis showed a pulmonary nodule, diffuse lymphadenopathy and hepatosplenomegaly. Cytopenias in sarcoidosis, when present, may reflect bone marrow infiltration. Hence, biopsy was obtained and bone marrow sarcoidosis was diagnosed. This manifestation, in spite of ethnic and gender predilection, is rarely seen. As with other forms of sarcoidosis, treatment comprises of corticosteroids.
Abbreviations: ANCA: Antineutrophil cytoplasmic antibody; BM: Bone marrow; BMS: Bone marrow sarcoidosis; CT: Computerized tomography; HIV: Human immunodeficiency virus; HLA: Human leukocyte antigen; MRI: Magnetic resonance imaging
... Sarcoidosis-lymphoma syndrome is characterized by the development of lymphoproliferative disorders following the diagnosis of sarcoidosis, with Hodgkin's lymphoma being the most common type [3,4]. Sarcoidosis association with monoclonal gammopathy of undetermined significance (MGUS) is a rare finding with only 10 cases reported until date [5][6][7][8][9][10][11][12][13]. ...
Sarcoidosis is a systemic inflammatory disease characterized by the presence of noncaseating granulomas in different organs. Sarcoidosis associated with monoclonal gammopathy of undetermined significance (MGUS) is a rare finding with only 10 cases reported to date. We describe a 79-year-old male patient who presented with dry mouth for 4 months. Lip biopsy done prior to admission showed nonnecrotizing epithelioid cell granulomas. On admission, laboratory analysis was significant for elevated calcium, decreased parathyroid hormone, increased erythrocyte sedimentation rate, undetectable parathyroid hormone-related peptide (PTHrp), mildly decreased 25-hydroxyvitamin D, elevated 1,25-dihydroxyvitamin D, elevated angiotensin converting enzyme, and positive Bence Jones protein in the urine. Serum protein electrophoresis showed an elevated gamma globulin level at 38% and an IgG monoclonal gammopathy with an M-spike of 1.47. Bone marrow biopsy was consistent with MGUS. The patient showed significant improvement with steroids and was discharged with close follow-up from nephrology and oncology. Salivary gland involvement in patients with sarcoidosis is a rare finding. Our case is a valuable addition to the small number of cases described in the literature supporting an association between plasma cell disorders and sarcoidosis. Larger prospective studies are needed to determine if a true association between the two diseases exists.
