ABSTRACT
Introduction: Diagnosis of AL amyloidosis requires demonstration of amyloid in affected tissues along with clonal plasma cells in bone marrow or presence of monoclonal light chains in blood. With the availability of serum light chain assay and immunophenotyping by flow cytometry, more cases of AL amyloidosis are being diagnosed. Here we present our experience of AL amyloidosis diagnosis and treatment in the era of modern diagnostics and therapy. Aims & Objectives: We aimed to describe the clinical presentations, laboratory features, treatment and outcomes of patients with AL amyloidosis in a single center using standard diagnostic tests and treatment with novel agents. Material(s) and Method(s): A retrospective analysis of AL amyloidosis patients, diagnosed in our hospital, a tertiary care center from January 2016 to June 2022 was conducted. The data was collected from departmental database. All statistical analyses were done by SPSS version 17. Result(s): Diagnosis of AL amyloidosis was done in 31 patients. Median age of presentation was 61 years. 25 (80.6%) were males. Major symptoms were pedal edema (38.7%) and shortness of breath (32.3%). Twenty four (77.4%) presented with ECOG PS >= 2. Most common systems involved were cardiac (54.8%) and renal (54.8%). Fourteen (45.2%) had two or more systems involvement while 17 (54.8%) had single system involvement. Lambda monoclonal light chain was present in 83.9% and kappa monoclonal light chain in 16.1%. Median M-protein was 0.59 g/dL (range 0-2 g/dL) and median bone marrow plasma cells were 6% (range-1-18%). Eighteen patients were treated;cyclophosphamide, bortezomib and dexamethasone (CyBORD) in 12/18 (66.7%) and bortezomib + dexamethasone in 6/18 (33.3%). Among 18 patients followed up with median follow up of 9 months (range 1-64 months), six expired;three due to COVID, two due to cardiac arrhythmia (during first cycle) and one due to relapse and rest 12 were alive. Among the 12 patients who were alive 6 were in complete hematological response. Conclusion(s): Our study presents the spectrum of clinical manifestations, management and outcomes of primary amyloidosis in Indian context. There is a need to increase the awareness among the physicians about amyloidosis so that early diagnosis can be made and timely treatment can be done with novel agents to improve the dismal historical results.
ABSTRACT
Introduction: Untreated/refractory severe aplastic anemia (SAA) is associated with very high mortality. Allogenic bone marrow transplantation or immunosuppressive therapy remains mainstay of treatment but these treatments are timely available to only a select subset of patients. Recently eltrombopag has been approved for treatment of SAA. Aims & Objectives: We aimed to describe clinical profile and treatment response in patients with SAA from a tertiary care centre. Material(s) and Method(s): A retrospective analysis of patients diagnosed with SAA over a period of 7 years from January 2015-December 2021 was performed. The details of demographic profile, laboratory features, treatment given and response were analyzed. Result(s): Ninety patients were diagnosed with SAA during this period out of which 18 patients went elsewhere for treatment. Seventy-two patients who received treatment in our hospital were included in the analysis. Sixty-two patients were SAA while 10 VSAA. PNH screening was done in 24 patients, out of which 17 (70%) had small clone. The details of treatment and response achieved is shown in Table 1. Eight patients (11.1%) received matched related donor allogenic hemopoietic cell transplant, out of which one had rejection followed by auto recovery while one died 6 months later due to covid 19 disease. Sixty-four patients received immunosuppressive therapy, forty-nine (76%) responded. Recurrence of SAA occurred in two patients who has achieved complete response to ATG therapy;one received second course of horse ATG + CSA + ETP and responded again. Conclusion(s): Timely diagnosis and appropriate treatment selection is of utmost importance to achieve optimal outcome in severe aplastic anemia. Eltrombopag has become an important addition not only in front line but also in relapsed refractory aplastic anemia. Patients lacking donor, or resources for ATG should be treated with cyclosporine and eltrombopag as early as possible. (Table Presented).
ABSTRACT
Background: Diagnosis of AL amyloidosis requires demonstration of amyloid in affected tissues along with clonal plasma cells in bone marrow or presence of monoclonal light chains in blood. With increasing awareness among physicians and availability of proper diagnostics, more cases of AL amyloidosis are being diagnosed. Here we present our experience of AL amyloidosis diagnosis and treatment in the era of modern diagnostics and therapy with novel agents. Aims: We aimed to describe the clinical presentations, laboratory features and outcomes of patients with AL amyloidosis in a single center using standard diagnostic tests and treatment with novel agents. Methods: A retrospective analysis of AL amyloidosis patients, diagnosed in our hospital, a tertiary care center in India from January 2016 to December 2021. The data was collected from departmental database. All statistical analyses were done by SPSS version 17. Results: Diagnosis of AL amyloidosis was done in 27 patients. Median age of presentation was 59 years. 22 (81.5%) were males. Major symptoms were pedal edema (37%), shortness of breath (22.2%), frothy urine (11.1%) and fatigue (11.1%). Twenty two (81.5%) presented with ECOG PS ≥ 2. Most common system involved was renal in 16 (59.2%), followed by cardiac in 13 (48.1%) and gastro-intestinal in 9 (33.3%). Fifteen (55.6%) had two or more system involvement while 12 (44.4%) had single system involvement. Lambda monoclonal light chain was present in 22/27 (81.5%) and kappa monoclonal light chain was present in 5/27 (18.5%). Median Hb was 11.6 g/dl (range 6.7- 14.8 g/dl), median M-protein was 0.69 g/dL (range 0-2 g/dL) and median bone marrow plasma cells were 7% (range- 1-18%). Fourteen patients were treated;cyclophosphamide, bortezomib and dexamethasone (CyBORD) in 10/14 (71.4%) and bortezomib + dexamethasone in 4/14 (28.6%). Among 14 patients followed up with median follow up of 13 months (range 6-60 months), 5 expired;3 due to COVID, one due to cardiac arrhythmia (during first cycle) and one due to relapse and rest 9 were alive. Among the 9 patients who were alive 6 were in complete hematological response and 3 were in partial response after 6 cycles of therapy. Summary/Conclusion: Our study presents the spectrum of clinical manifestations, management and outcomes of primary amyloidosis in Indian context. There is a need to increase the awareness among the physicians about amyloidosis so that early diagnosis can be made and timely treatment can be done with novel agents to improve the dismal historical results.
ABSTRACT
Introduction: A reduced absolute lymphocyte count in peripheralblood along with relative increase in neutrophil count has beenobserved consistently in hospitalized COVID-19 patients. The role ofbaseline lymphocyte subsets in COVID-19 is still unknown.Aims &Objectives: We aimed at analyzing the baseline lymphocytesubsets in COVID-19 patients and its impact on the outcome andseverity of the disease.Materials &Methods: Study was conducted retrospectively fromhospital electronic records. Diagnosis of COVID-19 disease wasbased on the RTPCR for SARS-COV-2 virus. Lymphocyte subsetswere determined using flowcytometry in COVID-19 patients onadmission to COVID ward. The variation in the baseline lymphocytesubsets according to the severity and outcome of the disease wasanalyzed.Result: Patients who died of COVID-19 disease had higher mean Blymphocytes and NK cells than the survivors but was not statisticallysignificant. T lymphocyte counts and CD8 + T cell counts showedstatistically significant (p< 0.05) reduction in patients who expiredthan who survived COVID-19 disease.Conclusions: We concluded that low CD8 + T cell counts atadmission may be predictive of patient outcomes in COVID-19.