scholarly journals A Prospective Observational Study of Clinico-Pathological Features, Prognostic Factors and Treatment Response to Primary Therapy in Multiple Myeloma at a Tertiary Care Centre

Blood ◽  
2019 ◽  
Vol 134 (Supplement_1) ◽  
pp. 5559-5559
Author(s):  
Saurabh Mishra ◽  
Ashok K Vaid ◽  
Nitin Sood ◽  
Manorama Bhargava ◽  
Bhuvan Chugh ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Treatment Response ◽  
Light Chain ◽  
Prospective Observational Study ◽  
Tertiary Care ◽  
High Dose ◽  
Primary Therapy ◽  
Lambda Light Chain ◽  
Tertiary Care Centre ◽  
Cell Support

Title: "A prospective observational study of clinico-pathological features, prognostic factors and treatment response to primary therapy in Multiple Myeloma at a tertiary care centre" Background: Multiple myeloma (MM) is characterised by the neoplastic proliferation of plasma cells leading to excess monoclonal immunoglobulins. The incidence of multiple myeloma in India ranges from 1.2 to 1.8 per 100,000. There is paucity of cytogenetic data from this part of subcontinent. The aim of our study is to report various clinicopathological features, evaluate biological markers of prognostication including cytogenetic variables and assess treatment response after standard primary therapy. Methods and Materials: The study was carried out at a tertiary care center in Northern India. After final diagnosis of multiple myeloma was established, each patient was risk stratified via FISH cytogenetic analysis as per the revised ISS. Definitive management plan was individualised, including assessment for high dose therapy with peripheral blood stem cell support. Treatment response were recorded as per standard IMWG response criteria. Significant toxicities associated with treatment were also recorded. Results: Eighty consecutive patients with newly diagnosed multiple myeloma were enrolled prospectively from April 2017 to November 2018. The median age at diagnosis was 63 years, and number of males & females were equal. The most common presenting symptom was back pain (67.8 % patients) and most frequent clinical sign was pallor (86.4 %). All four CRAB features were documented only in 16.9% patients. M-Band was present in 96.6% patients and on SFLC assay 59.3% and 40.7% were kappa and lambda light chain restricted respectively. Most common heavy chain abnormality detected was IgG. Seventy percent patients had lytic lesions on imaging while only 3% suffered skeletal related events. Cytogenetic evaluation by interphase FISH was carried out in all patients upfront. No chromosomal abnormality was documented in 61.25 % while among those with chromosomal abnormalities, most commonly detected was del13q14.3 (23.75 %). Overall, 66.6% patients were stratified as standard risk, 28.1% as intermediate risk and only 3 (5.3%) patients were categorised as high risk. Most common induction regimen was VRD. Overall response rate was 94.9 % and VGPR or better responses were observed in 77.9% patients. Most common adverse effect of therapy was peripheral neuropathy of all grades. Of the 77 patients who completed primary therapy, 21.4% patients underwent high dose therapy with peripheral blood stem cell support while 67.7% patients were started on maintenance therapy. Four (5.1 %) non-responder was started on 2nd line treatment. On univariate analysis, higher deeper responses (VGPR or better) were observed in patients with IgA & IgG related myeloma and better overall response rates were seen in IgG related myeloma. Those with kappa light chain myeloma had 5.67 times higher likelihood of achieving response as compared to lambda light chain myeloma. Also, patients with kappa light chain myeloma achieved higher VGPRs as compared to lambda light chain myeloma. There was 17 times high risk of non-response in the presence of local bony tenderness. None of the findings were found to be significant on multivariate analysis. Conclusions: Use of interphase FISH to identify various cytogenetic markers help in stratification & staging of the disease which in turn act as a marker for prognostication. They should be a part of standard care in multiple myeloma. In majority we could administer treatment in accordance to standard practice guidelines and response rates were similar to those reported my seminal studies. Our study in a longer follow up will yield some useful information which will help in the better care of the patients. Disclosures No relevant conflicts of interest to declare.

IgD Myeloma: Clinical Features and Outcome In The Era Of Novel Agents

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 1935-1935 ◽  
Author(s):  
Flora Zagouri ◽  
Efstathios Kastritis ◽  
Argiris S. Symeonidis ◽  
Nikolaos Giannakoulas ◽  
Eirini Katodritou ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
High Risk ◽  
Renal Dysfunction ◽  
Light Chain ◽  
The Other ◽  
Novel Agents ◽  
Primary Therapy ◽  
Lambda Light Chain ◽  
Prognostic Features ◽  
High Risk Disease

Abstract IgD multiple myeloma is a rare variant of the disease and in various series accounts for about 2% of patients with symptomatic myeloma. It has been suggested that patients with IgD myeloma may have an inferior outcome when compared to other patients. However, data on IgD myeloma patients treated in the novel agent era are lacking. In order to assess the frequency and evaluate the outcome and the specific characteristics of patients with IgD myeloma we analyzed the database of the Greek Myeloma Study Group to identify such patients. Between January 2000 and December 2012, among the 1239 patients with previously untreated, symptomatic, myeloma, 31 patients (2.5%) were diagnosed with IgD myeloma. Of interest, 84% of patients with IgD myeloma had lambda light chain (versus 38% of the patients with other subtypes of MM, p<0.001). The median age of patients with IgD myeloma was 65 years (range 26-80 years) versus 68 years (range 23-96 years) for patients with other subtypes of MM (p=0.073), while 10% of patients with IgD versus 28% of the other patients were >65 years (p=0.023) and 10% of patients with IgD versus 3.8% of the other patients were ≤40 years of age. Patients with IgD myeloma presented more often with significant renal dysfunction (serum creatinine ≥2 mg/dl in 52% versus in 19%, p<0.001 or eGFR <30 ml/min/1.73m2 in 42% versus 18% in patients of other subtypes, p=0.001) and excreted larger amounts of Bence Jones protein (59% excreted ≥2 gr per day versus 17% of the other patients, p<0.001). Patients with IgD myeloma had also more often features of high risk disease including ISS-3 disease (60% versus 37% for the other patients, p=0.011) and elevated serum LDH (≥300 IU/L) in 29% versus10% of the other patients (p=0.001). Response to primary therapy for patients with IgD myeloma was similar to other patients (at least PR in 77% versus 72% respectively), although there was a trend for better quality of responses in patients with IgD myeloma (sCR and CR in 26% versus 15% of patients, and at least VGPR in 53% versus 29% in patients of other subtypes, respectively, p=0.059). However, more patients with IgD myeloma had received primary therapy with bortezomib-based regimens (40% versus 22%) and less often IMiD-based therapy (20% versus 35%), while similar proportions of patients received conventional chemotherapy-based regimens (40% versus 44%; p=0.043). Despite the increased frequency of features of high risk disease in patients with IgD myeloma, the median survival of these patients was 51.5 months versus 50.7 months for patients of other subtypes (p=0.850). In a multivariate model to adjust for differences in prognostic features, IgD myeloma was not associated with a different prognosis. We also compared the outcome of patients with IgD myeloma treated before and after January 1st, 2000. The survival of the patients with IgD myeloma who started therapy before versus after 2000 was 44 months (p=0.018). In conclusion, in a large series of patients with symptomatic multiple myeloma, the incidence of IgD myeloma is 2.5%. The vast majority of patients with IgD myeloma is associated with lambda light chain and present more often with significant Bence Jones proteinuria, significant renal dysfunction and features of advanced disease. However, their outcome in the era of novel agents is similar to that of patients with other myeloma subtypes. Disclosures: No relevant conflicts of interest to declare.

Assessment of Free Light Chains in the Ascites and Pleural Effusion in Patients with Multiple Myeloma: A Report of 2 Cases

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 5078-5078
Author(s):  
Sawsan Rashdan ◽  
Sherif Farag ◽  
Rafat Abonour ◽  
Attaya Suvannasankha
Keyword(s):  
Multiple Myeloma ◽  
Pleural Fluid ◽  
Light Chain ◽  
High Dose Chemotherapy ◽  
High Dose ◽  
Lambda Light Chain ◽  
Bodily Fluids ◽  
Flow Cytometric ◽  
Cat Scan ◽  
Serum And Urine

Abstract Abstract 5078 A recently developed free light chain (FLC) immunoassay has made possible the quantitative measurement of kappa and lambda FLCs by automated nephelometry. Significant clinical evidence indicates the benefit of serum FLCs in the diagnosis of monoclonal gammopathies, AL amyloidosis and nonsecretory MM patients missed by conventional electrophoretic methods. Reference and diagnostic intervals for FLCs have been developed for serum and urine analyses. FLCs in other bodily fluids may provide a valuable tool in other conditions. For example, kappa FLCs are frequently elevated in the cerebrospinal fluid of patients with clinically suggested multiple sclerosis, and therefore can assist in confirming the diagnosis. Analysis of other bodily fluids for FLCs posts theoretical concerns which relate to a lack of standard reference, as well as, the plausible interference of both cellular and protein content in the fluid with data interpretation. We report the use of FLC levels in discerning the etiology of recurrent ascite and pleural effusion in 2 patients with a known diagnosis of multiple myeloma. Samples were centrifuged prior to analysis. Other steps of sample processing were based on a standard serum protocol. FLC was analyzed using the FreeLite™ assay (Binding Site) on Beckman Coulter Image analyser. Patient #1: A 64 year old man was diagnosed with IgG kappa multiple myeloma which was in complete remission according to the International Myeloma Working Group criteria after an induction therapy with lenalisomide and dexamethasone followed by high dose chemotherapy and stem cell transplantation. He presented with abdominal distention and progressive weight loss. A CAT scan of the abdomen showed moderate amount of ascites with no other detectable pathology. Ascite fluid analysis showed 200 nucleated cells/mm3. Differential counts included 8% neutrophils, 30% monocytes, 61% lymphocytes and 1% eosinophils. Serum-ascitic albumin gradient was 0.9 gm/dl. No atypical cells were seen by histological or flow cytometric analyses. Cultures for bacteria and fungi were negative. Serum CA19-9, CEA, AFP and PSA were normal. Serum and protein electrophoresis did not show M spike. Serum kappa FLC and lambda FLC were 10.5 and 6.97 ug/ml, serum K/L FLC was 1.51. Kappa FLC and lambda FLC in ascetic fluid was 12.1 and 8.4ug/ml, respectively. Peritoneoscopy with a peritoneal biopsy showed nodular deposits on the peritoneal lining and pathology confirmed a poorly differentiated adenocarcinoma. Patient #2: A 62-year old lady was diagnosed with lambda light chain multiple myeloma when she presented with transfusion dependent anemia and renal failure. She was treated with la enalidomide and dexamethasone combination and had stable disease. Her treatment regimen was changed to a bortezomib, liposomal doxorubicin and dexamethasone combination, which she progressed on. The patient was treated with salvage combination of D-PACE as a bridge to high dose chemotherapy and stem cell transplantation. One week after treatment, she developed progressive shortness of breath and confusion. She was noted to be hypoxic at room air and had decreased breath sound on the right side of her chest. Laboratory tests were significant for WBC of 0.3x 103/mm3. Chest X-ray and CAT scan of the chest showed large amount of pleural fluid with lower lobe atelectasis, and cardiomegaly. An echocardiogram showed a left ventricular ejection fraction of 40% with no wall motion abnormality. The patient was treated empirically with broad spectrum antibiotics and dieresis. The amount of pleural fluid did not improve in 48 hours after treatment. Thoracentesis was performed and pleural fluid kappa and lambda FLCs were <3.00 mg/L and 3221 mg/L respectively. Subsequent cytology and flow cytometric analysis confirmed a monoclonal plasma cell population that expresses CD138, CD38, CD56 and lambda light chain. Conclusion: We report that FLC analysis of other bodily fluids, aside from serum and urine, is feasible and may provide complimentary value to other histologic and biochemical analyses especially in discerning whether the etiology for the fluid accumulation related to the underlying multiple myeloma. Disclosures: Abonour: Celgene: Membership on an entity's Board of Directors or advisory committees.

A STUDY OF THE CLINICAL PROFILE OF PATIENTS WITH HYPOSPADIAS AT A TERTIARY CARE CENTRE AT KANPUR, INDIA

2021 ◽  
pp. 65-66
Author(s):  
R. K. Maurya ◽  
Shraddha Verma ◽  
R. K. Tripathi ◽  
Amit Yadav
Keyword(s):  
Prospective Observational Study ◽  
Undescended Testis ◽  
Tertiary Care ◽  
Care Centre ◽  
Common Site ◽  
Tertiary Care Centre ◽  
Medical College ◽  
Treatment History ◽  
Congenital Hernia ◽  
Distal Hypospadias

Background: Hypospadias is a common congenital anomaly in which the anterior urethra is incompletely developed and does not extend to the tip of the glans penis. The present study was aimed to study the clinical prole of hypospadias. Settings and Design:This was a prospective, observational study. Methods: This prospective study was conducted at Department of General Surgery, LLR & Associated Hospitals, GSVM Medical College, Kanpur, India, from January 2019 to October 2020, on 72 patients of hypospadias, after taking clearance from the Institutional Ethical Committee, and taking proper informed consent for participation. Data collected for each patient included age, sex, demography, symptoms with duration, and relevant past and treatment history. Results: The most common age of presentation was between 1-5years of age (48.61%, n=35). Distal hypospadias (DH) was the most common site. (47.22%, n=34). 4.16% (n=3) patients presented with urethrocutaneous stula. Chordee was more commonly seen in patients with mid & proximal penile hypospadias (64.70%, n=33). In the present study, 4.1% (n=3) had associated undescended testis, 4.1% (n=3) had an associated bid scrotum and 1.3% (n=1) had an associated congenital hernia. Conclusions: Most cases of hypospadias present before 10 years of age. Distal hypospadias is the most common type. Chordee is associated most commonly with proximal penile and mid penile hypospadias. It may also be associated with other congenital anomalies like, undescended testis, bid scrotum or congenital hernias

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