|Year : 2021 | Volume
| Issue : 4 | Page : 189-191
Multiple myeloma presented with renal failure in a patient with familial Mediterranean fever: Presentation of a rare association
Samar Tharwat1, Mohammed Kamal Nassar2
1 Internal Medicine Department, Rheumatology and Immunology Unit, Mansoura University, Mansoura, Egypt
2 Internal Medicine Department, Mansoura University Nephrology and Dialysis Unit, Mansoura University, Mansoura, Egypt
|Date of Submission||27-May-2021|
|Date of Decision||15-Jun-2021|
|Date of Acceptance||06-Jul-2021|
|Date of Web Publication||21-Aug-2021|
Prof. Samar Tharwat
Mansoura University Hospital, El Gomhouria St, Mansoura, Dakahlia Governorate
Source of Support: None, Conflict of Interest: None
Familial Mediterranean fever (FMF) is a hereditary auto-inflammatory disorder characterised by repeated attacks of fever associated with polyserositis. The association between multiple myeloma (MM) and FMF has been rarely reported. Here, we describe a 39-year-old male with a long history of FMF presenting with acute kidney injury that has been proved to be caused by myeloma cast nephropathy. To the best of our knowledge, this is the fourth case to be reported with such association in the literature.
Keywords: Familial Mediterranean fever, multiple myeloma, renal failure
|How to cite this article:|
Tharwat S, Nassar MK. Multiple myeloma presented with renal failure in a patient with familial Mediterranean fever: Presentation of a rare association. Curr Med Res Pract 2021;11:189-91
|How to cite this URL:|
Tharwat S, Nassar MK. Multiple myeloma presented with renal failure in a patient with familial Mediterranean fever: Presentation of a rare association. Curr Med Res Pract [serial online] 2021 [cited 2021 Sep 25];11:189-91. Available from: http://www.cmrpjournal.org/text.asp?2021/11/4/189/324251
| Introduction|| |
Familial Mediterranean fever More Details (FMF) is the most common auto-inflammatory disease. It is characterised by sporadic self-limited attacks of fever associated with serosal inflammation. The disease is associated with mutations in the MEFV gene-encoding pyrin that causes excessive inflammatory reactions through uncontrolled production of interleukin-1. Colchicine remains the main treatment of FMF, and the objective of treatment should be to prevent acute attacks and amyloidosis.
Multiple myeloma (MM) is a specific form of haematological malignancies, with around 114,000 new cases worldwide per year. It is the second most common adult hematologic malignancy, and the skeleton is the most commonly affected site. The association between MM and FMF has been rarely reported. This paper aims to discuss an exuberant case of a long history of FMF presenting with renal failure which was found to be caused by myeloma cast nephropathy. Further investigations confirmed the diagnosis of MM.
| Case Report|| |
A 39-year-old male was diagnosed with FMF 10 years earlier. The patient reported recurrent episodes of fever accompanied by abdominal and chest pain, significantly reduced by a daily dose of colchicine of 1.5 mg over the past 10 years.
On March 2020, the patient presented to the outpatient nephrology clinic complaining of gradually reduced amount of urine over the past month. On examination, he looked restless and anxious, not dehydrated or icteric. There was remarkable pallor with heart rate of 80 beats/min, respiratory rate of 18/min, temperature of 37.2°C and blood pressure of 170/100 mmHg. Systemic examination was unremarkable except for bilateral oedema of the lower limbs and hepatosplenomegaly.
Upon investigations, complete blood count revealed haemoglobin, WBCs and platelets count of 7.6 g/dl, 8.4 × 109/L and 374 × 109/L, respectively. The serum levels of creatinine, albumin and calcium were 11.4 mg/dl, 1.85 g/dl and 9.3 mg/dl, respectively. The erythrocyte sedimentation rate was markedly elevated (140 mm/h) and 24-h urinary protein excretion was 2 g/day. Liver enzyme and total bilirubin were within normal range. Serological tests for HCV, HBV and HIV were negative. The chest X-ray was found to be normal. Abdominal ultrasound showed hepatosplenomegally and both kidneys were normal. Renal biopsy was performed and revealed no glomerular abnormality, and the Congo red stain was negative. Interstitial fibrosis has been detected in more than 50% of the biopsied tissue and the renal tubules displayed an acute tubular injury with PAS negative casts surrounded by giant cells; a picture suggestive of myeloma cast nephropathy with no evidence of amyloidosis [Figure 1]. Serum protein electrophoresis showed increase in the beta-globulin fraction with a band of concentration (5.18 g/dl) suggesting monoclonal gammopathy [Figure 2]. Bone marrow aspirate was performed and demonstrated a heavy (>90%) infiltration by plasma cells. Flow cytometry and immunophenotyping were suggestive of MM as these cells showed positive expression for CD38, CD138 and CD56 and negative expression for CD27 and CD19. A skeletal survey revealed two well-defined lytic bone lesions, at the frontal bone of the skull and the body of third lumbar vertebra. The final diagnosis was MM in a male patient with FMF. Haemodialysis was initiated in view of uremic symptoms, and the patient was referred to the haematology outpatients' clinics and chemotherapy was started.
|Figure 1: Renal biopsy shows acute tubular necrosis with myeloma cast nephropathy: (a) tubular necrosis with no abnormalities in glomerular basement membranes, cellularity or mesangium, (b) interstitial fibrosis of more than 50% of tissue biopsied, (c) acute tubular injury with PAS negative casts surrounded by giant cells|
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|Figure 2: Serum protein electrophoresis shows monoclonal band in the beta-globulin region|
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| Discussion|| |
We presented a case of a middle-aged male with a 10-year history of FMF who developed renal failure as a first presentation of MM. To the best of our knowledge, the association between MM and FMF is rarely reported in the world literature; only three cases with such association have been reported.,,
FMF is the most common auto-inflammatory disorder. Clinically, it is characterised by recurrent attacks of fever and serositis, often triggered by multiple environmental factors. Renal dysfunction is amongst the most debilitating complications of FMF. Renal dysfunction may be related to secondary amyloidosis or to other unusual non-amyloid glomerular diseases such as IgG and IgM mesangial proliferative glomerulonephritis and IgA nephropathy.,
MM is one of the most common haematological malignancies. In patients with newly diagnosed MM, approximately one-third will present with estimated glomerular filtration rates <30 mL/min/1.73 m2 at the time of diagnosis.
The pathogenesis of MM development in patients with FMF is unknown; however, it may be related to underlying immune-mediated mechanism or colchicine toxicity. In general, there is no evidence of increased incidence of cancer in FMF males under 50 years of age. Older studies have identified a few cases of mesothelioma in FMF patients with no history of asbestos exposure, with speculation that repeated peritoneal or pleural inflammatory attacks may predispose to their growth. Some reports indicated a co-occurrence of FMF and a group of malignancies, leading to the conclusion that FMF may be associated with increased risk of cancer. Although there is no reported increase in FMF-related haematological cancers, the probability that heterozygous MEFV variants may be risk factors has been raised with overrepresentation in Turkish studies of myelodysplastic syndrome, myeloma and acute myeloid and lymphoid leukaemia. Dysregulation of interleukin (IL-)1α or IL-1β production contributes to the pathogenesis of numerous auto-inflammatory disorders including FMF. IL-1α has also emerged in recent years as an important cytokine in cancer pathogenesis. Moreover, colchicine, the main treatment of FMF, is a neutral, liposoluble alkaloid that interferes with growth and mitosis of the microtubules. Colchicine blocks mitosis and has been linked with haematological complications and leukaemia in rare cases.
In the end, MM and FMF may have a common pathogenic pathway. This case highlights the association between MM with FMF and shades of light on non-amyloid kidney diseases that may occur in FMF patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that their names and initial will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]