First Manifestation Of Multiple Myeloma Research Articles

Case Report: Spontaneous pneumothorax revealing multiple myeloma: a case report

Various causes like trauma, infection, pulmonary disease or neoplasm can lead to spontaneous pneumothorax. We report a rare case of a spontaneous pneumothorax as first manifestation of multiple myeloma. A 58-year-old patient presented suffering from dyspnea and right-sided chest pain, with no history of trauma. On examination, the patient had bilateral rib tenderness. The respiratory rate was 30 breaths/min and oxygen saturation was 88%. The chest physical exam revealed unequal breath sounds, an hyperresonance with percussion and decreased wall movement on the right side. The analysis of arterial blood gas revealed hypoxemia (arterial oxygen tension: 7.59 kPa) and hypercapnia (arterial carbon dioxide tension: 5.99 kPa). Laboratory data showed a raised C reactive protein level (133.8 mg/L), hyper-calcemia (serum calcium: 12.18 mg/dL) and a decreased plasma albumin level (31.9 g/L). Chest radiography and thoracic computed tomography revealed multiple ribs and sternum fractures leading to a partial pneumothorax on the right side. Subsequent workup for multiple myeloma showed elevated levels of immunoglobulin. Results of initial laboratory tests revealed an IgG gamma paraprotein, a urine protein electrophoresis of 1450 mg/24 hours and a β-2 microglobulin rate of 3.35. The diagnosis of multiple myeloma was confirmed with a bone marrow infiltration of 20% of atypical plasmatic cells. Cytogenetic investigations did not show any chromosomal abnormalities, especially the t (4,14) translocation. The patient was diagnosed with multiple myeloma stage IIIA according to Durie–Salmon classification. Appropriate treatment with oxygen therapy and systemic analgesic was started, associated with a cure of zoledronic acid in order to decrease the calcium level. The evolution was characterized by the complete resolution of the pneumothorax in 7 days and the normalization of the calcium level. The autologous stem cell transplant was the treatment of choice for this patient.

Cerebral Infarction as the First Manifestation of Multiple Myeloma and AL Amyloidosis: a Case Report

Cerebral Infarction as the First Manifestation of Multiple Myeloma and AL Amyloidosis: a Case Report

Cold Agglutinin Disease in Non-Secretory Multiple Myeloma: A Case Report

Skin involvement is not common as the first manifestation of Multiple Myeloma (MM). Although extremely rare, leucocytoclastic vasculitis, plasmasytoma, autoimmune bullous disease, livedo reticularis and Raynaud’s phenomenon may be the initial presentation of MM. Raynaud’s phenomenon and livedo reticularis related to cold exposure can be due to Cold Agglutinin Disease (CAD) or cryoglobulinemia and can be seen as the first manifestation of MM. In this case study, we described a 55-year-old man complaining of limbs livedo reticularis and Raynaud’s phenomenon during cold weather. Further evaluations revealed anemia and elevated ESR. Skull X-ray showed multiple punched-out lesions. Finally, serum protein electrophoresis and bone marrow aspiration confirmed the diagnosis of non-secretory MM as the underlying disease of CAD. The patient was referred to the hematologist for the treatment of MM.

Successfully non-surgical management of flail chest as first manifestation of multiple myeloma: A case report

Successfully non-surgical management of flail chest as first manifestation of multiple myeloma: A case report

Pemphigus vulgaris as the first manifestation of multiple myeloma: a case report

BackgroundThe association between pemphigus and malignancy has been well documented for decades but an association between pemphigus vulgaris and multiple myeloma is unusual. We report a case of pemphigus vulgaris revealing multiple myeloma.Case presentationA 55-year-old Malagasy man, with no significant past medical history, presented with bullous and erosive skin lesions involving his trunk and scalp for the last 2 months. He had no mucous membrane involvement. A diagnosis of pemphigus vulgaris was made on skin biopsy and direct immunofluorescence of perilesional skin revealing immunoglobulin G deposition in the intercellular spaces in the epidermis. In an enzyme-linked immunosorbent assay, his serum autoantibody index against desmoglein-1 and 3 was found to be 112 RU/mL and 34 RU/mL respectively. Serum immunoelectrophoresis showed a monoclonal gammopathy with a markedly elevated immunoglobulin G level (2880 mg/dL) in association with a lambda free light chain. Bone marrow aspirate showed 6% plasma cell infiltration. Further investigations, including creatinine blood test and whole body radiographic examinations, showed that he had initially clinical stage I multiple myeloma of the immunoglobulin G-λ type. Six months later, bone tomography revealed vertebral compression fractures of the thoracic and lumbar spine that correlated with his back pain topographically. Anti-myeloma treatment including melphalan and prednisone led to an immediate decline in monoclonal immunoglobulin G concentration. Skin and hematologic remission were maintained for 12 months.ConclusionsAbsence of mucosal involvement, lack of vacuolar degeneration at the interface, and absence of apoptotic, dyskeratotic keratinocytes ruled out paraneoplastic pemphigus in our case. Pemphigus vulgaris should be considered even if possible underlying disease for which paraneoplastic pemphigus is recognized is present.

Prevalence and clinical significance of abnormal serum kappa/lambda light chain ratio in patients with chronic kidney disease.

A range of malignant diseases (both solid organ tumors and hematogenous malignancies) may manifest as proteinuria/nephrotic syndrome. Multiple myeloma (MM) typically involves the kidneys and proteinuria, and chronic kidney disease (CKD) may also be the first manifestation of MM. The aim of the study was to evaluate the prevalence and clinical significance of abnormal serum free kappa/lambda light chain ratio in patients referred to a renal center for a diagnostic workup for proteinuria or CKD of an unknown origin (or both). Free light chain tests were performed in 92 consecutive patients (mean age, 63 ±13.9 years; women, 38%; men, 62%) using FreeLite kits. In addition, serum creatinine, calcium, and albumin levels as well as estimated glomerular filtration rate (eGFR) were measured and blood count and proteinuria were assessed. In 39 patients, kidney biopsy was also performed. Nephrotic syndrome was found in 38% of the patients; nonnephrotic proteinuria, in 39.1%; and isolated reduction of eGFR (without proteinuria), in 22.9%. MM was confirmed in 5 patients (5.43%; all patients had a highly abnormal kappa/lambda ratio). After the exclusion of the subjects with MM, the abnormal kappa/lambda ratio was found in 37 patients (42.5%); MM was excluded in all patients after careful hematological evaluation, including bone marrow biopsy in 12 cases. The percentage of the abnormal kappa/lambda ratio was high regardless of the proteinuria level, eGFR, or the type of glomerulopathy diagnosed by kidney biopsy. The kappa/ lambda ratio did not correlate with age, proteinuria, or eGFR. Our results suggest that an abnormal kappa/lambda ratio (in most cases higher than normal) is a common and, most likely, nonspecific finding in patients with proteinuria or CKD of an unknown origin (or both).

A Rare Case of Sinonasal Extramedullary Plasmacytoma with Orbital Involvement

ABSTRACT Extramedullary plasmacytoma (EMP) is a rare entity in the head and neck region. It is phenotypically and immunologically different from multiple myeloma. Chronic transformation to multiple myeloma or its disseminated form is well known. It may even be the first manifestation of multiple myeloma also popularly known as secondary EMP. We present a 43-year-old male patient with history of persistent progressive nasal obstruction with occasional epistaxis. Endoscopy revealed a right sinonasal mass which on excision biopsy was consistent with features of EMP. Extramedullary plasmacytoma is a rare differential diagnosis for sinonasal mass. However, multiple myeloma should be ruled out via bone marrow examination, bone scan and serum and urine electrophoretic evaluation before arising at a diagnosis of EMP. In spite of EMP of nasal cavity being a rare entity, it should be considered as one of the rare differential diagnoses. How to cite this article Ashish G, Chandrasekharan R, Parmar H. A Rare Case of Sinonasal Extramedullary Plasmacytoma with Orbital Involvement. Int J Head Neck Surg 2015; 6(3):121-124.

Oral lesion as unusual first manifestation of multiple myeloma: case reports and review of the literature.

Extramedullary plasmacytoma (EMP) and solitary bone plasmacytoma (SBP) represent a disease continuum through a multistage process of cell differentiation, survival, proliferation, and dissemination, strictly related to multiple myeloma (MM), the second most common hematological malignancy. Herein, we report two cases of recurrent oral plasmacytoma progressed to MM, in which the first clinical sign of a more widespread disease was limited to the mouth. Based on our experience, we recommend a strict workup for the differential diagnosis between EMP, SBP, and MM for patients with oral plasmacytoma, including radiological exam of the skeleton, magnetic resonance imaging (MRI) of the bone, and positive emission tomography (FDG-PET). MRI and possibly PET can all be used to more sensitively detect EM plasmacytoma sites.

Maxillary pain is the first indication of the presence of multiple myeloma: A case report

Multiple myeloma is a primary malignancy of bone marrow characterized by the clonal proliferation of plasma cells and production of monoclonal immunoglobulin. The disease occurs more frequently in males, with the average age at diagnosis being ∼60 years. The first manifestation of multiple myeloma is varied and depends on the sites and extent of involvement. The predominant clinical symptoms of multiple myeloma are associated with bone pain and renal dysfunction. Neoplastic cells usually produce large amounts of monoclonal immunoglobulin light or heavy chains that can be detected in serum or urine, while plasmacytoma may be identified on marrow biopsy. The present study reported on the case of a 69-year-old male patient presenting with a complaint of a painful lesion in the left maxilla. Physical examination, imaging, laboratory investigations and biopsy were conducted, confirming the diagnosis of multiple myeloma. The results obtained suggest that the dentist should address oral manifestations as first indications of multiple myeloma.

Thrombotic thrombocytopenic purpura as initial and major presentation of multiple myeloma

To the Editor, Thrombotic thrombocytopenic purpura (TTP) is a rare but fatal disease characterized by microangiopathic hemolytic anemia, thrombocytopenia, neurologic disturbances, renal abnormalities and fever. It is now recognized that congenital and acute acquired TTP are due to a deficiency of von Willebrand factor (vWF) cleaving protein, also known as ADAMTS13, which inhibits vWF-dependent platelet aggregation [1]. Acquired thrombotic microangiopathy comprises about 40 % of all cases of TTP, including pregnancy, drugs, HIV infection, hematopoietic stem cell transplantation, autoimmune diseases or malignancies. The malignancies associated with TTP are usually solid tumors. TTP as first manifestation of multiple myeloma is very rare. To our knowledge, there is only one documented case in the literature [2]. Here we report a case of multiple myeloma, which initially presented with TTP. A 61-year-old male presented to local county hospital with a 2-month history of fatigue, jaundice and chest pain. Laboratory tests showed white blood cell of 4.9 9 10/L, hemoglobin (Hb) of 74 g/L, platelet count of 53 9 10/L, total bilirubin (TBIL) of 100.1 lmol/L (reference range 5.0–16.7 lmol/L) and direct bilirubin (DBIL) of 11.8 lmol/ L (0–5.7 lmol/L). On the third day after admission, he developed confusion, disorientation and delirium at night. Subsequently, he was transferred to our hospital for further diagnosis and treatment on December 8, 2010. Physical examination showed pale face, jaundice and somnolent with loss of orientation. No focal neurological deficit was present. Laboratory tests showed Hb of 75 g/L, platelet count of 52 9 10/L, reticulocytes of 11 %, TBIL of 103.9 lmol/L (reference range 5.1–17.1 lmol/L), DBIL of 51.8 lmol/L (0.0–6.0 lmol/L), serum lactate dehydrogenase (LDH) of 447.8l/L(reference range 109.0–245.0l/L) and blood urea nitrogen (BUN) of 8.2 mmol/L (reference range 2.90–7.14 mmol/L), creatinine(Cr) of 144.8 mmol/L (reference range 40.0–133.0 mmol/L). Serum protein electrophoresis was normal. Serum immunoglobulins, calcium and urinary Bence Jones protein was in normal range. The coagulation tests (prothrombin time, activated partial thromboplastin time, fibrinogens) and blood ammonia were within normal limits. Direct Coomb’s test was negative. Tests for HIV, Hepatitis B, Hepatitis C and autoimmune serologies were negative. Cranial computed tomography and magnetic resonance imaging remained normal. Bone marrow aspiration showed increased plasma cells of 50 % with matureappearing. His blood smear displayed schistocytes of 64/1,000. ADAMTS-13 activity was 29.9 % (reference 40–140 %). A diagnosis of TTP was made. Plasmapheresis was emergently initiated followed by fresh frozen plasma infusions of 300 mL daily with concomitant 10 mg intravenous dexamethasone per day for 6 days, then the patient was on regimen of oral prednisone of 45 mg per day with a gradual reduction. Dramatic response was observed after three plasma exchanges with 1.5 times of total plasma volumes. Clearness of mind, improvement in his chest pain, reticulocytes decreased to 3.9 % and TBIL, DBIL, LDH became normal. His platelet count was 70 9 10/L, and Hb was 80 g/L. Because the presence of plasma cell infiltration in the patient’s bone marrow biopsy, the diagnosis of multiple myeloma was highly suspected. Tests for monoclonal paraprotein were repeated performed, and the results were Xiang Xiao and Hai-ying Zhong contributed equality to this study.

Pulmonary Embolism as the First Manifestation of Multiple Myeloma

Multiple myeloma is considered a hypercoagulable state due to several mechanisms such as the increased IL-6 and immunoglobulins production, the defective fibrinolytic mechanism, and the acquired resistance to activated protein C that are involved in the pathogenesis and clinical futures of the disease. We describe a case of a female patient who presented to the hospital with pulmonary embolism as the first manifestation of the hypercoagulability of multiple myeloma.

UNUSUAL MANIFESTATION OF MULTIPLE MYELOMA: FOCAL AFFECTION OF CENTRAL NERVOUS SYSTEM IN A PATIENT WITH CHRONIC LYMPHOCYTIC LEUKAEMIA

Involvement of the central nervous system as the first manifestation of multiple myeloma is very rare. To present an unusual case of the primomanifestation of a multiple myeloma in the form of a focal affection of the central nervous system in a patient with chronic lymphocytic leukaemia. A female patient diagnosed with chronic B-lymphocytic leukaemia with gradually increasing right-sided cerebellar symptomatology. The CT examination revealed expansion of the cranial vault with significant compression of brain structures. The tumour was extirpated and the histological examination led to a diagnosis of a plasmocytic myeloma. A thorough examination confirmed the diagnosis of multiple myeloma with significant osteolytic involvement of the skeleton. A combined chemo- and radiotherapy resulted in adjustment in the focal neurological finding, and a partial remission of the multiple myeloma was achieved. The above presented case describes two very unusual states: the primomanifestation of a multiple myeloma in form of a focal affection of the central nervous system, and the coincidence of a multiple myeloma as the second haematological malignancy in a patient with chronic B-lymphocytic leukaemia.

Myelomatous Pleural Effusion of Multiple Myeloma: Characteristics and Outcome.

Abstract 3874Poster Board III-810 IntroductionMyelomatous pleural effusion (MPE) in multiple myeloma (MM) is rare (<1%). As most reported MPEs are in the form of case reports, the characteristics of MPE are unclear. We report 30 MM patients presenting with MPE, including 11 cases as the first clinical manifestation of MM. To the best of our knowledge, this is the largest retrospective study ever reported about MPE. Patients and MethodsMulticenter retrospective data of MM patients presenting MPE, who were diagnosed from January 1998 to June 2009 were analyzed. Diagnosis of MPE was based on positive cytology or biopsy in conjunction with M protein by protein electrophoresis or immunofixation in pleural effusion. ResultsThe median age was 53 (range 21-78). Twenty patients were male and 10 were female. The myeloma isotype was IgG in 8, IgA in 7, IgD in 5, light chain in 9 and non-secretory myeloma in 1 patient. Most patients had high-risk disease based on initial D-S stage IIIA or IIIB (83%). Thirteen of 19 patients, who had available BM chromosomal analysis or FISH result, showed various karyotypic abnormalities including deletion of chromosome-13 (n=8). MPE was the first manifestation of MM in 11 patients (early-onset group). In the other 19 patients (late-onset group), MPE developed at a median of 20 months (range 1 – 75) from diagnosis of MM. The effusions were left-sided in 6, right-sided in 9 and bilateral in 15 patients. In more than half of the cases, a thoracic skeletal lesion, lung parenchymal lesion, pleural involvement or mediastinal lymphadenopathy was concomitantly present. Before the development of MPE, median 2 lines of chemotherapy (range 1 – 9) were applied in late-onset group. Seven patients of this group received high-dose chemotherapy with stem cell transplantation (6 autologous and 1 allogeneic stem cell). After onset of MPE, the median number of chemo-regimen was 1 (range 0 -3) in both groups and 3 patients got high-dose chemotherapy with autologous stem cell transplantation (1 in early-onset and 2 in late-onset group). After onset of MPE, total 27 chemo-regimens were applied to 19 patients and the regimens were based on VAD (vincristine, adriamycin, dexamethasone) in 12, thalidomide in 5, bortezomib in 4 and other regimen in 6. Pleurodesis was done in 2 patients. MPE disappeared in 11 patients and median response duration, defined as days from disappearance to relapse of MPE or death of any cause, was 123 days (95% confidence interval (CI): 10 – 236). Median survival was 66 days (95% CI: 29 – 103) from onset of MPE and 16 months (95% CI: 5 – 28) from initial diagnosis of MM. From onset of MPE, median survival was 84 (95% CI: 57 – 111) and 55 days (95% CI: 0 – 113, p=1.0) in early and late-onset group, respectively. From initial diagnosis of MM, median overall survival was 2.8 (95% CI: 2.4 – 3.2) and 26 months (95% CI: 12 – 40, p<0.001) in early and late-onset group, respectively. Causes of death were progression of disease, progression with concomitant infection or infection (50, 23 and 8 %, respectively). ConclusionsBase on this analysis, MPE is a poor prognostic indicator, irrespective of the time of onset. When pleural effusions develop in a patient with MM, clinicians should perform prompt and appropriate diagnostic work-up. In the case of MPE, aggressive treatment including novel agents or autologous stem cell transplantation should be considered, especially if MPE is the initially presenting manifestation of MM. Further evaluation of the utility of various novel chemotherapeutic agents in the setting of MPE is needed to improve treatment outcome. Disclosures:No relevant conflicts of interest to declare.

Percutaneous vertebroplasty at C2: case report of a patient with multiple myeloma and a literature review

Percutaneous vertebroplasty (PVP) of the axis is a challenging procedure which may be performed by a percutaneous or a transoral approach. There are few reports of PVP at the C2 level. We report a case of unstable C2 fracture treated with the percutaneous approach. The fracture was the first manifestation of multiple myeloma in a previously healthy 47-year-old woman. After local radiotherapy and chemotherapy, the fracture was still unstable and the patient had been continuously wearing a stiff cervical collar for 9 months. Complication-free PVP resulted in pain relief and stabilization and use of the cervical collar could be discontinued. At 18 months follow-up the patient remained free from pain, the fracture was stable and she had returned to work. The purpose of this article is to present the technical facts and to highlight the benefits and potential complications of the procedure. The technical characteristics of the procedure, the indication and results of the present case are discussed together with previously reported cases of PVP treatment at C2.

Intrasellar plasmacytoma presenting as a non-functional invasive pituitary macro-adenoma: Case Report &amp; Literature Review

We report an uncommon case of an intrasellar plasmacytoma presenting as a non-functional invasive pituitary macro-adenoma as the first manifestation of multiple myeloma. A 57 year old woman was referred to our department with a presumed diagnosis of a non-functioning pituitary macro-adenoma. She reported a 3 month history of headaches and a 2 week history of sudden onset of right facial numbness. Preoperative endocrine evaluation was remarkable only for a modestly elevated serum prolactin. A magnetic resonance imaging (MRI) scan revealed 3.6 x 5 x 4 cm enhancing homogeneous intrasellar mass with extension into the sphenoid and cavernous sinuses bilaterally; the optic chiasm was not displaced. She underwent transphenoidal surgery of the sellar lesion. The surgical specimen was heavily infiltrated with abnormal plasma cells, which stained almost exclusively for Kappa light chain immunoglobulins. An extensive investigation was undertaken to look for occult myelomatous disease. A diagnosis of multiple myeloma was made 1 month later based on a combination of clinical, pathological and radiologic features. She underwent radiation therapy directed towards the residual sellar tumor, followed by chemotherapy and autologous stem cell transplantation. Review of the world literature revealed only 22 previous reports of patients in whom a solitary plasmacytoma or multiple myeloma first presented as a sellar mass; in all cases mimicking clinically and radiologically a non-functioning invasive pituitary adenoma however with additional cranial nerve involvement. Intrasellar plasma cell tumors are rare tumors which may mimic non-functioning invasive pituitary tumors. The diagnosis should be suspected in patients with well preserved anterior pituitary function and cranial nerve neuropathies in the presence of significant sellar destruction.

Paresthesia of the left lower lip as a first manifestation of multiple myeloma —A case report

We experienced a case of multiple myeloma in a 62-year-old woman who visited our hospital with a chief complaint of paresthesia in the left lower lip, which was described as numb chin syndrome, as her initial symptom.

Multiple Myeloma and Hairy Cell Leukemia: A Rare Association or Coincidence?

Hairy cell leukemia (HCL) and multiple myeloma (MM) are well-defined entities with distinctive clinical and pathological features. Although most cases of HCL and MM fit their classic descriptions, more recent studies have revealed that their clinical and morphological boundaries may not only overlap but a transformation of HCL into MM could also occur. We report another case of HCL followed by the development of MM after 9 years. He also developed hemarthrosis of his right ankle at the time of diagnosis of MM. PCR analysis of DNA extracted from the bone marrow aspirate was negative for the presence of a monoclonally rearranged immunoglobulin heavy chain gene. Immunophenoytping revealed no evidence of HCL. There are several possible explanations for the development of MM in HCL patients, such as the coexistence of separate disease entities or different clinical and morphologic phases of a single disease entity. An accurate diagnosis of HCL or MM is critical because of differences in their treatment. Hemarthrosis in this patient may also have been the first manifestation of MM, a feature of MM which has rarely been reported.

Extramedullary Plasmacytoma of the Orbit as Initial Presentation of Multiple Myeloma

This report is of a patient with a rare extramedullary plasmacytoma of the orbit as the initial manifestation of systemic multiple myeloma. A 34-year-old otherwise healthy man presented with periorbital pain for 2 weeks with proptosis, diplopia, and limited ocular motility in the left eye. Magnetic resonance imaging showed an extraconal mass at the superotemporal region of the left orbit with bony erosion and an enhancing orbital lesion in the temporal quadrant of the right orbit. Histopathological examination of the orbital mass revealed a plasmacytoma with lambda light chain restriction. Systemic evaluation revealed an immunoglobulin G myeloma with multiple myeloma in the first lumbar vertebra, hypercalcaemia, and renal impairment. Chemotherapy was planned. Plasmacytoma of the orbit is rare and can present as the first manifestation of multiple myeloma. Ophthalmologists should be aware of such a presentation and must act promptly. This report reviews the presenting features.

RADIATION THERAPY FOR PARAPLEGIA DUE TO MULTIPLE MYELOMA.

SPINAL CORD compression has been reported to occur in 6% to 18% of patients with multiple myeloma. 1,2 Since paraplegia may be the first manifestation of multiple myeloma, it is important to consider this diagnosis in every patient with cord compression. Paraplegia in multiple myeloma has frequently been regarded as a strong indication for surgical intervention. 3,4 The successful use of radiation therapy alone has been occasionally reported 3 but not sufficiently stressed. It is the purpose of this report to reemphasize the potentialities of the latter approach in this clinical situation. Report of Cases Case 1.— A 54-year-old Negro male was admitted on June 29, 1960, because of backaches of 11 months' duration and weakness and numbness of the lower extremities which began two weeks prior to admission and progressed rapidly. On admission he was well developed and well nourished. The blood pressure was 125/95 mm Hg. He had