Should patients with renal failure associated with myeloma be dialysed?

In a study of renal function in multiple myeloma seven patients presented with renal failure and three developed it 16-106 months after diagnosis. All were dialysed. Infection with dehydration was a precipitating factor in all seven cases of acute or acute on chronic renal failure. Of these, two patients recovered normal renal function and one other was left with permanent renal impairment but no longer required dialysis. Results from the seven patients with acute renal failure and for the three with more chronic features support the practice of dialysis for all patients who present with renal failure. Dialysis is not indicated for those patients with progressive myelomatous disease. The study showed no evidence that chemotherapy permitted recovery from established renal failure. The prognosis in this elderly group is heavily dependent on the presence of cardiovascular or other degenerative disease.     

The Effect of Urography on Renal Function in Patients with Multiple Myeloma

Reports in the medical literature of seven patients with multiple myeloma who died of acute renal failure following intravenous urography prompted a study of 39 patients with multiple myeloma who were subjected to intravenous urography at the Cleveland Clinic from 1940 to 1959. Four developed acute renal failure and two died within three weeks. All four revealed evidence of renal damage, or insufficiency, or both prior to urography. Thirty-five patients, 15 of whom had renal damage, had no untoward reaction to intravenous urography. These observations suggest that urography is associated with a small but definite risk in patients who have multiple myeloma and renal involvement.     

IgD Plasma Cell Neoplasia: Clinical Manifestations and Characteristic Features

Three patients, one with plasma cell leukemia and clinically asymptomatic hypernephroma and meningioma, and two others with multiple myeloma, had M-components of IgD/λ type. In the first case, IgD globulin was found in the serum, ascitic and pleural fluids. Including our patients, 50 cases of IgD myeloma have been reported in the literature. A review of this group showed some significant differences from the other classes of multiple myeloma. IgD myeloma seems to involve a larger proportion of younger people, 66% being less than 59 years of age. The involvement of internal organs and renal damage were more frequent in IgD myeloma than in other classes. Serum total protein was frequently not increased, the relative concentration of M-component was often low and in 12% there was no spike in electrophoresis. The diagnosis therefore was sometimes difficult. In a quarter of the cases Bence Jones proteinemia was found and in 15% there were multiple spikes, both these manifestations being rare in IgG or IgA classes of myeloma. In 89%, IgD globulin had lambda type light chain, clearly contrasting with the figure of approximately 30% in other classes. Bence Jones protein was found in the urine in 91%. The survival time seemed to be shorter than in other myelomas.     

Inadequate erythropoietin production (epo) in patients with multiple myeloma

Anaemia is the most common haematological complication in patients with malignant diseases. It is found in 60%-90% of cases with multiple myeloma. The pathogenesis of this hypoproliferative, normochromic, normocytic anaemia is complex. Results from clinical studies which evaluate the efficacy of recombinant human erythropoietin (rHuEpo) refer to the possibility that patients with multiple myeloma independently of renal function could have Epo deficiency. Based on this finding, the aim of the study was to evaluate the erythropoietin production in patients with multiple myeloma in order to define clinical conditions of Epo deficiency and thereby enable rational use of this expensive drug. 42 patients with multiple myeloma were examined. The control group consisted of 25 patients with iron deficiency anaemia. 14 healthy volunteers represented the so-called "normal" control. The adequacy of Epo production was estimated from the graphic representation of the linear regression between Epo and haemoglobin (Hb) in the control group, as well as from O/PEpo ratio as a measure of the degree of adequacy of Epo production (O -- observed Epo value, P -- predicted Epo value from the regression equation of the control group). The erythropoietic activity was estimated from the graphic representation of the linear regression between soluble transferin receptors (sTfR) and Hb in the control group, as well as from O/PsTfR ratio, as a measure of the degree of adequacy of erythropoietic activity (O -- observed sTfR value, P -- predicted sTfR value from the regression equation of the control group). Significant inverse correlation between Epo and Hb was found in patients with multiple myeloma but preserved renal function, which was not the case in patients with renal insufficiency. 43% of patients without renal insufficiency and 85% of patients with renal insufficiency had inadequate Epo response to anaemia. In both patient groups (with and without renal insufficiency) instead of the expected inverse relationship between Hb and sTfR as in the control group, a positive correlation was found. 76% of patients had inadequate sTfR response to anaemia. There is a positive correlation between O/PEpo and O/PsTfR which is in favour of Epo driven erythropoiesis. O/PEpo and O/PsTfR in patients with multiple myeloma are significantly lower in comparison to the control group, which also points to the inadequacy of erythropoietin production, respectively erythropoietic activity. In conclusion, the results from this study show unambiguously that anaemia in patients with multiple myeloma appears because of decreased erythropoiesis as a consequence of bone marrow infiltration with malignant plasma cells as well as inadequate Epo production. Most probably, there are two forms of inadequate Epo production: one because of the renal insufficiency and the other that found in patients with anaemia of chronic diseases the mechanism of which is not clear.     

多发性骨髓瘤并发上消化道侵袭性念珠菌病2例报道及文献复习

目的 探讨多发性骨髓瘤(Multiple myeloma,MM)并发上消化道侵袭性念珠菌病的临床特点,提高诊治该病的水平.方法 回顾分析我科2例多发性骨髓瘤并发上消化道侵袭性念珠菌病的诊治过程,分析、总结治疗经验,并结合文献复习骨髓瘤并发上消化道侵袭性念珠菌感染的易感因素、临床表现、诊断和鉴别诊断及治疗.结果 MM患者1,女性,异基因造血干细胞移植术后4个月发生腹泻伴腹部隐痛,抗移植物抗宿主病(graftversus host disease,GVHD)治疗无效,经胃镜诊断上消化道念珠菌病,予卡泊芬净治愈出院.MM患者2,男性,化疗过程中疾病进展,并出现腹胀、腹痛,对症处理改善,再次化疗时上述症状加重并发肺部念珠菌病,发生急性心、肾功能不全,经胃镜诊断上消化道念珠菌病,予伊曲康唑、米卡芬净治疗,最终因肺部真菌感染加重,治疗无效死亡.结论 多发性骨髓瘤并发上消化道侵袭性念珠菌病易发生误诊、漏诊.临床医生需提高对该病的认识,首选电子胃镜明确诊断、判断疗效.     

米卡芬净与伊曲康唑治疗多发性骨髓瘤并发侵袭性真菌病的疗效和成本分析

目的 比较米卡芬净与伊曲康唑在多发性骨髓瘤并发侵袭性真菌病（IFD）病例治疗中的疗效及成本.方法 采用回顾性分析方法,入选2010年1月～2012年11月我科发生侵袭性真菌病的37例多发性骨髓瘤患者的临床资料.对比分析米卡芬净治疗组与伊曲康唑治疗组两组患者的疗效、治疗时间、治疗成本及不良反应.结果 米卡芬净组17例,伊曲康唑组20例,米卡芬净组治疗有效率为76.46％ （13/17）,伊曲康唑组治疗有效率70％ （14/20）,差异无统计学意义（P=0.42 ＞0.05）.米卡芬净组2例发生消化道不适反应.伊曲康唑组3例发生消化道不适反应,2例低钾血症并消化道不适反应;3例患者出现药物性肾功能损害,l例停药后肾功能恢复,2例出现急性肾功能损害.米卡芬净组不良反应发生率为11.76％ （2/17）,伊曲康唑不良发生率为40％ （8/20）,两组差异有统计学意义（P＜0.05）.以治疗第14天总有效率作为疗效判断标准,米卡芬净组和伊曲康唑组成本疗效比分别为263.73和289.11.结论 米卡芬净与伊曲康唑对骨髓瘤合并IFD的治疗有效率差异无统计学意义,不良反应差异有统计学意义,前者成本效益比优于后者.     

Intravenous infusion of pituitary adenylate cyclase-activating polypeptide (PACAP) in a patient with multiple myeloma and myeloma kidney: a case study

We have recently shown significant renoprotective effects with the administration of pituitary adenylate cyclase-activating polypeptide (PACAP) in models of myeloma kidney. PACAP markedly inhibited the production of proinflammatory cytokines stimulated by immunoglobulin light chains in human renal proximal tubule epithelial cells and in the kidneys of rats infused with myeloma light chains. PACAP was also shown to suppress the proliferation of human kappa and lambda light chain-secreting multiple myeloma-derived cells. In this case study, an 81-year-old male patient with active multiple myeloma and myeloma kidney was infused intravenously with synthetic human PACAP38 at a rate of 4 pmol/kg/min for 120 min. The continuous infusion increased the level of PACAP38 in blood, with a plateau at about 0.2 nM during the infusion. The level of PACAP in the blood rapidly declined after the cessation of administration with a half-life of about 5-10 min. The continuous infusion did not significantly alter the basal glucose level, blood gases or blood pressure. There was a large reduction in free lambda light chains in urine after the start of the treatment with PACAP. These studies show that PACAP can be safely used in humans and suggest that it could be used as a novel therapeutic agent for the treatment of multiple myeloma and myeloma kidney.     

Clinical application of a rapid technique for urinary electrophoresis in multiple myeloma

Proteinuria in three cases of multiple myeloma was studied without prior concentration of the urine, using a simple technique of urinary protein electrophoresis. In each case a free light chain spike was observed in association with a glomerular, tubular or normal urine protein electrophoresis pattem. The suggestion is made that this simple procedure may become a screening test for investigation of renal diseases.     

Volunteer and clinical studies with carfecillin: a new orally administered ester of carbenicillin.

Blood and urine levels of carbenicillin were measured in 10 healthy volunteers and four patients with renal failure after single and multiple oral dose of carfecillin. Urinary levels after 1000-mg doses in healthy subjects were considered sufficient for treatment of Pseudomonas aeruginosa urinary infections, but the serum levels were too low for chemotherapy of systemic infections with this organism even in severe renal failure. Urinary infections were treated in 35 inpatients with a seven-day course of carfecillin. The infection was eradicated in 21 cases (60%). In 12 cases the pathogen was Ps. aeruginosa, which was eradicated from eight patients (67%). Many patients had severe urinary tract disease. Side effects were virtually absent.     

Clinical features of common acute lymphoblastic leukemia antigen (CALLA)-positive myeloma: report of four cases

Four patients with common acute lymphoblastic leukemia antigen (CALLA)-positive myeloma are presented. The subclasses of monoclonal protein were IgD kappa (1 case), IgA lambda (1 case), and IgA kappa (2 cases). Bence Jones proteinuria was seen in all cases. The clinical stages were determined as IIA (2 cases) and IIIA (2 cases). All patients died with a median survival time after diagnosis of 62 days due to rapid development of renal failure (3 cases), and renal insufficiency and pneumonia (1 case). According to light microscopic evaluation, these myelomas corresponded to plasmablastic (1 case), immature (2 cases), and intermediate (1 case) types. Both CALLA and a cytoplasmic immunoglobulin identical with the serum monoclonal protein were simultaneously detected in single cells from all cases using immunofluorescent double labeling. These findings suggest that CALLA-positive and plasma-blastic myelomas constitute clinically a subgroup characterized by extremely poor survival but they represent cytologically different subcategories.     

Frequent association of idiopathic myelofibrosis with plasma cell dyscrasias

In a retrospective analysis of 199 cases of myeloproliferative diseases a concomitant plasma cell dyscrasia was found in three out of 46 patients with idiopathic myelofibrosis. Chronic myeloid leukemia, polycythemia vera or unclassifiable myeloproliferative disorders were in no case associated with monoclonal gammopathy. One patient with idiopathic myelofibrosis had primarily coexistent IgG-lambda paraproteinemia and increasing osteolytic lesions; histologic evidence of multiple myeloma, however, was insufficient. In the second patient the interval between diagnosis of idiopathic myelofibrosis and IgG-kappa paraproteinemia was 11 years. After a stable period of 9 years' duration the paraprotein level rapidly increased, associated with depression of normal background immunoglobulins and progressive bone marrow failure. The exact nature of this patient's malignant plasma cell dyscrasia remained uncertain. In the third case benign monoclonal gammopathy of the IgM-lambda type was diagnosed 13 years after idiopathic myelofibrosis. A review of the literature confirms a remarkably high incidence of monoclonal gammopathies in idiopathic myelofibrosis. Benign monoclonal gammopathy seems to occur in at least 8% of the patients while only a few cases of concomitant multiple myeloma have been reported. It may be speculated that plasma cell dyscrasias in idiopathic myelofibrosis reflect involvement of the lymphoid lineage in the neoplastic stem cell disorder.     

Plasmacytoma with involvement of the urinary bladder. Report of a case diagnosed by urine cytology

BACKGROUND: Plasmacytoma of the bladder is a rare but important entity. We report a case of plasmacytoma of the bladder that was diagnosed by urinary cytology. CASE: A 71-year-old male with a history of multiple myeloma presented in renal failure. Renal ultrasound revealed right-sided, moderate hydronephrosis with a 4 x 4-cm, posterolateral, obstructing mass. Magnetic resonance imaging demonstrated a bladder mass involving the bladder base, right lateral wall and dome with extension into the perivesical tissues on the right. The mass showed a moderate degree of enhancement following intravenous gadolinium administration. Urine cytology was performed to evaluate for bladder carcinoma or other malignancies besides plasmacytoma. The specimen was signed out as multiple myeloma of the bladder. Cystoscopy and biopsy were subsequently performed on the bladder mass. The diagnosis of plasmocytoma was made, confirming the urine cytology diagnosis. CONCLUSION: Urinary cytology can be a diagnostic tool for plasmocytoma involving the bladder.     

Acute Renal Failure and Open Heart Surgery

A retrospective study of 428 open heart operations showed the incidence of mild and severe renal failure to be 26% and 4·7% respectively. The mortality rate was 38% in the mild cases and 70% in the severe cases. Only half of the patients whose death was associated with renal failure showed macroscopic or microscopic renal lesions at necropsy. The patients who developed renal impairment had significantly higher mean preoperative blood urea (40 mg/100 ml) than the non-renal-failure cases (33 mg/100 ml). Periods of perfusion over 60 minutes, mean perfusion pressures below 80 mm Hg, and multiple valve replacement operations also increased the incidence of renal failure. There was no statistical correlation between the age of individual patients, the degree of cooling, and postoperative blood urea values. There was no evidence to suggest that frusemide or mannitol separately or together influenced the development of renal failure. Peritoneal dialysis was preferred for initial treatment of patients with severe renal failure, and haemodialysis was required only in special cases.     

Multiple myeloma associated with multilobated plasma cell nuclei

Ten cases of multiple myeloma are reported in which there were a large number of plasma cells with excessively convoluted or lobulated nuclei. These cases represent 3% of the 297 evaluable multiple myeloma patients treated at our institution over a 22-year period. All 10 had intermediate or advanced stage disease at the time of diagnosis, and all have died after a mean survival of 19.5 months. Ultrastructural features of 2 cases are described. When found in abundance, such cells can cause difficulty in establishing a morphologic diagnosis of multiple myeloma because of their resemblance to other cell types. Therefore, it may be necessary to perform immunoperoxidase staining and/or electron microscopy to confirm the plasmacytic identity of these cells. The findings add further support to the contention that the presence of excessive nuclear convolutions is not a completely reliable indication of T-cell, as opposed to B-cell, lineage.     

蝮蛇咬伤10885例救治分析

目的：探讨提高蝮蛇咬伤的救治水平。方法,回性总结我院１９７２－１９９９年蝮蛇咬伤患者１０８８５年,采用抗蝮蛇毒血清结合中药蛇伤合剂治疗;对合并急性呼吸衰竭的８５例患者,早期气管切开保持气管畅通,同时正压人工呼吸;对合并急性肾功能衰竭的１０３例患者,早期碱化尿液,应用足量利尿剂,必须时进行腹膜透析;对合并急性心功能衰竭的４３例患者,在应用强心、利尿、扩血管药物的同时,早期静脉给予山莨菪碱（６５４－２     

Plasmacytoma of the breast. Unusual initial presentation of myeloma: report of two cases and review of the literature

Two patients who presented initially with breast masses proven to be plasmacytomas, are described. In both cases the breast masses led to the diagnosis of multiple myeloma, which was retrospectively already present at the time of the breast biopsy. One patient with IgG lambda-type myeloma developed plasma cell leukemia and amyloidosis and failed to respond to radiochemotherapy. The second patient was in complete remission, four years after therapy, but has subsequently relapsed and died. All cases of breast plasmacytoma, described in the medical literatures are reviewed, and guidelines for future therapy are suggested.     

Plasma cell tumours: cytomorphological features in a series of 12 cases diagnosed on fine needle aspiration cytology

U. Handa, S. Chhabra and H. Mohan
Plasma cell tumours: cytomorphological features in a series of 12 cases diagnosed on fine needle aspiration cytology Objective: Plasma cell tumours represent autonomous proliferation of plasma cells and can manifest as multiple myeloma, monoclonal gammopathy of undetermined significance, variants of plasma cell myeloma or plasmacytoma. Methods: We report 12 cases of plasma cell tumours, which were initially diagnosed as plasmacytoma on fine needle aspiration cytology (FNAC). The patients were further subjected to bone marrow examination, serum electrophoresis, urine examination for Bence–Jones proteins, and x‐ray examination of the skeleton. Results: The cytological smears from all cases were cellular and showed numerous plasma cells in varying degrees of maturity. Subsequent to investigations, five cases were labelled as multiple myeloma with secondary extramedullary plasmacytoma, three as solitary bone plasmacytoma and two as primary extramedullary plasmacytoma. In the remaining two cases, bone marrow and urine examination findings were not available, so a conclusive diagnosis of multiple myeloma or solitary plasmacytoma could not be made. Conclusion: The study highlights the role of FNAC in the diagnosis of plasma cell tumours. Subsequent work‐up and follow‐up of these patients is important to rule out the presence of multiple myeloma.     

68例多发性骨髓瘤的临床特点及实验室检查回顾性分析

目的：探讨68例多发性骨髓瘤MM患者的临床首发症状,实验室相关检查特点。方法：回顾分析我院2002年1月-2012年3月期间68例MM患者临床表现及实验室相关资料。结果：68例MM患者中临床表现具有多样性,细胞形态学检查中骨髓瘤细胞比例大于20％的占76％,骨髓瘤细胞比例小于20％的占24％,骨髓中成熟红细胞呈缗钱状排列者占78％,血清蛋白电泳中53例患者出现M蛋白,免疫球蛋白IgG增高15例,IgA增高11例,轻链型4例,X线检查结果48例提示骨质有异常改变。结论：临床表现和实验室检查对明确诊断MM及早期治疗都有很重要的意义。     

Breast myeloma: a report of 3 cases with fine needle aspiration cytologic findings

BACKGROUND: Multiple myeloma of the breast is very rare, and the fine needle aspiration (FNA) findings have not been reported before. CASES: Two cases of multiple myeloma presented with bilateral breast nodules during treatment with chemotherapy. One case of multiple myeloma presented initially with a left breast mass. FNA smears of all 3 cases revealed numerous plasma cells, plasmablasts and multinucleated giant plasma cells. The smears were diagnosed as plasma cell tumors. Serum immunoelectrophoresis revealed IgG myeloma in 2 cases and IgA myeloma in 1. Marrow aspirates revealed > 30% plasma cells. Two patients died, and 1 was alive at this writing. CONCLUSION: The aspiration cytology findings of myeloma can be confuse, with primary and secondary tumors of the breast. The previous clinical history and ancillary studies, such as bone marrow study and serum immunoelectrophoresis, are essential to the correct diagnosis.     

唑来膦酸注射液联合经皮椎体成形术治疗脊柱多发性骨髓瘤的临床疗效

目的:观察脊柱多发性骨髓瘤行唑来膦酸注射液与经皮椎体成形术联合治疗的临床疗效。方法:2010年8月至2017年3月,16例行多发性骨髓瘤患者接受经皮椎体成形术与唑来膦酸注射液联合治疗,评定疗效、疼痛缓解及治疗前后血碱性磷酸酶、血清钙、生活质量,记录不良反应。结果:(1)疗效:完全缓解1例,部分缓解12例,微小缓解1例,疾病进展1例,无变化1例,总有效率为87.5%(14/16)。(2)疼痛缓解:显效9例,有效6例,无效1例。总有效率93.75%(15/16)。(3)血碱性磷酸酶、血清钙、生活质量:治疗后优于治疗前(P0.05)。(4)研究期间不良反应轻微。结论:脊柱多发性骨髓瘤行唑来膦酸注射液与经皮椎体成形术联合治疗有助于缓解疼痛和改善生活质量。