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Oral cavity cancer (OCC) essentially presents at a non-metastatic stage. Almost all patients are treated with a curative intent. Surgical approaches are the mainstay of treatment accompanied by radiotherapy and chemotherapy where applicable. This updated guideline emphasizes a personalized approach to the treatment of OCC, considering tumor characteristics, patient preferences, and the latest advancements in diagnostic methods. These guidelines aim to optimize treatment strategies, improve patient quality of life, and ensure adherence to the most current clinical practices in managing OCC, especially with respect to the Indian scenario.

Laryngeal cancer typically presents at a nonmetastatic stage, and the majority of patients are treated with curative intent. Due to the functional and cosmetic morbidities associated with surgical approaches, nonsurgical modalities are increasingly favored. This update to the guidelines emphasizes a multidisciplinary approach tailored to the Indian healthcare context, enhancing treatment strategies and optimizing patient outcomes. Key recommendations include the use of concurrent chemoradiotherapy and induction chemotherapy regimens, with a focus on organ preservation whenever feasible.

We provide the South Asian Declaration, containing the consensus guidelines for coronavirus disease 2019 (COVID-19) vaccination in cancer patients.

Background: The phase III MONALEESA-7 trial (NCT02278120) assessed ribociclib + endocrine therapy (ET) versus ET in premenopausal women with HR+/HER2− advanced breast cancer (ABC). The relationship between work productivity loss (WPL) and domains of European Organisation for Research and Treatment of Cancer Quality of Life (EORTC QLQ-C30) and the breast cancer (BC)-specific module (QLQ-BR23) has not been explored in ABC. In this post hoc analysis (data cutoff, November 30, 2018), we assessed the correlation between the WPL component of the Work Productivity and Activity Impairment: General Health (WPAI:GH) questionnaire and EORTC QLQ-C30/BR23 domains. Methods: We analyzed EORTC and WPAI:GH data from 329 patients in both treatment arms of MONALEESA-7 who were employed during the trial. Separate univariable mixed-model repeated measures (MMRM) regression models were fitted for each domain, with WPL as dependent variable and each EORTC domain score as a single fixed-effect covariate. Linear and quadratic relationships were considered based on the Akaike information criterion. Next, two separate multivariable MMRM regression models were fitted with WPL a dependent variable and all QLQ-C30/BR23 domain scores as fixed-effect covariates. The strength of correlation between WPL and EORTC domains was assessed in terms of minimally important differences for the QLQ-C30/BR23 modules. Results: Our univariable analysis showed that greater WPL was statistically significantly associated with lower levels of overall quality of life (QoL) and other functional domains and with higher levels of all symptomatic domains of the QLQ-C30/BR23 modules. Our multivariable analysis determined that this correlation was primarily driven by changes in QoL; physical, role, social, and future perspective domains; and BC-specific symptomatic domains. Conclusion: This analysis determined the QoL domains that correlate with WPL in premenopausal patients with HR+/HER2− ABC. These results may inform prognostic tools to identify and characterize patients with greater risk for WPL and help design interventional strategies to minimize WPL.

Next generation sequencing studies in Chronic lymphocytic leukemia (CLL) have revealed novel genetic variants that have been associated with disease characteristics and outcome. The aim of this study was to evaluate the prognostic value of recurrent molecular abnormalities in patients with CLL. Therefore, we assessed their incidences and associations with other clinical and genetic markers in the prospective multicenter COMPLEMENT1 trial (treatment naive patients not eligible for intensive treatment randomized to chlorambucil (CHL) vs. ofatumumab-CHL (O-CHL)). Baseline samples were available from 383 patients (85.6%) representative of the total trial cohort. Mutations were analyzed by amplicon-based targeted next generation sequencing (tNGS). In 52.2% of patients we found at least one mutation and the incidence was highest in NOTCH1 (17.0%), followed by SF3B1 (14.1%), ATM (11.7%), TP53 (10.2%), POT1 (7.0%), RPS15 (4.4%), FBXW7 (3.4%), MYD88 (2.6%) and BIRC3 (2.3%). While most mutations lacked prognostic significance, TP53 (HR2.02,p

De novo (pretreatment) epidermal growth factor receptor T790M mutation in non-small-cell lung cancer (NSCLC) is rare when detected by standard genotyping methods. We present a case of concurrent de novo T790M and L858R mutations detected by direct sequencing in treatment-naïve metastatic NSCLC. This case is worthy of mention as the presence of this mutation has a bearing on the choice of treatment. This article aims to evaluate the clinical outcome for metastatic NSCLC with de novo T790M mutation and formulate an optimum treatment plan in this clinical scenario. The novel targeted therapy agents have also been reviewed.

Background. Acute promyelocytic leukemia is characterized by t(15;17). This leads to the formation of PML/RARα which blocks the differentiation of blasts at the stage of promyelocytes. This is reversed by all-trans-retinoic acid (ATRA), a vitamin A derivative. Acute myocardial ischemia is a rare side effect of ATRA. Case Report. We report a case of acute coronary syndrome manifesting as an adverse effect of ATRA in a lady with APL who had no other risk factors for cardiovascular disease. Conclusions. We emphasize the need for high index of suspicion for the diagnosis of this entity. In the light of this case, the rare instances of ATRA associated acute myocardial ischemia recorded in the literature and the options available for treatment of acute promyelocytic leukemia sans ATRA have been reviewed.

Neuroendocrine neoplasms are defined as epithelial neoplasms with predominant neuroendocrine differentiation. They can arise in almost every organ of the body although they are most commonly found in the gastrointestinal tract and respiratory system. Nasal cavity and paranasal sinuses are a rare site for neuroendocrine carcinoma. In contrast to the other regions, neuroendocrine tumours of the sinuses have been reported to be recurrent and locally destructive. Very few cases of paranasal sinus neuroendocrine carcinoma have been reported till date. Difficulty in pathologic diagnosis and rarity of this malignancy have hindered the progress in understanding the clinical course and improving outcomes. We herein report a case of poorly differentiated neuroendocrine tumour of ethmoid and sphenoid sinus with invasion of orbit and intracranial extension. The patient had complete response at the end of chemoradiation and he was disease-free for 9 months duration after which he developed bone metastasis without regional recurrence.

Hypopharyngeal cancer usually presents at a locally advanced stage. Almost all patients are treated with a curative intent. As surgical approaches are functionally and cosmetically morbid, nonsurgical modalities are preferred. This review emphasizes a comprehensive diagnosis workflow, the integration of less toxic induction chemotherapy regimens, and a multidisciplinary approach to patient care. It aims to optimize treatment outcomes while preserving quality of life, particularly in the Indian healthcare context.

Oropharyngeal cancer typically presents at a nonmetastatic stage and is primarily treated with curative intent. While surgical options are available, they are often associated with significant functional and cosmetic morbidity, leading to a preference for nonsurgical modalities. The updated guidelines address the management of oropharyngeal squamous cell carcinoma in the Indian context, emphasizing the importance of a multidisciplinary approach. The guidelines detail diagnosis workflows, treatment protocols for early-stage and locally advanced cancers, and the role of human papillomavirus (HPV) status in guiding therapy. Special attention is given to emerging treatment deintensification strategies for HPV-associated cancers, aiming to optimize outcomes while minimizing treatment-related toxicity.

Head-and-neck squamous cell carcinoma (HNSCC) remains a significant public health concern, particularly in India, where its incidence is disproportionately high due to risk factors such as tobacco and alcohol use. The updated guidelines for the management of HNSCC reflect a comprehensive review of current evidence and practices tailored to the Indian context. They emphasize a multidisciplinary approach and the integration of novel therapeutic modalities, including immune checkpoint inhibitors and metronomic chemotherapy. Enhanced diagnostic protocols and a focus on supportive care underscore the commitment to improving patient outcomes and quality of life. This update aims to provide healthcare professionals with clear, evidence-based recommendations that address the unique challenges faced by patients with HNSCC in India.

Nasopharyngeal carcinoma (NPC) is a rare malignancy that poses significant treatment challenges, particularly in the context of curative intent. This updated guideline consolidates contemporary evidence and clinical practices tailored to the Indian healthcare landscape. It emphasizes a multidisciplinary approach to diagnosis and treatment, incorporating advances in chemotherapy, radiotherapy, and supportive care. Key recommendations include the use of induction chemotherapy for locoregionally advanced cases, detailed risk stratification, and specific management strategies for recurrent and metastatic disease, all aimed at improving patient outcomes.

Introduction: The chemotherapy schedules with cytotoxic dose or weekly regimes are still challenging, weighing the benefits versus toxicities. This prospective randomized study is an attempt to assess the efficacy of two schedules of cisplatin in management of locally advanced HNSCC. Objectives: The objectives of this study was to evaluate tolerance, tumour response and toxicities of concurrent chemoradiation with cisplatin in weekly and three weekly regimes. Methods: Locally advanced oropharyngeal squamous cell carcinoma patients fit for concurrent chemoradiation with cisplatin 40 mg/m2 (weekly) and 100 mg/m2 (3 weekly) were randomized to Arm A and B concurrently with radiotherapy of 70Gy/35frs/7 weeks. Statistical Analysis: Chi-square/ Fisher Exact test has been used to find the significance of study parameters on categorical scale between the groups. The statistical software SPSS 15.0 was used. Results: Between December 2010 and January 2013, 60 patients were enrolled. The median cycles of cisplatin in Arm-A was 5 and 2 in Arm-B. The complete response of 80.9% vs 75% and partial response of 14.3% vs 12.5% was observed in both arms respectively. There was no statistical difference in acute radiation and hematological toxicities between the two groups. With median follow up of 28 months, the 2 and 5 years overall survival was 55% and 58%; 41.6% and 32.3% in arms A and B respectively. Conclusion: In our study of locally advanced oropharyngeal carcinoma treated with radical radiotherapy comparing concurrent chemotherapy with cisplatin weekly vs 3 weekly had no significant difference in overall response, complete response and acute toxicities.

Background: Health-related quality of life (HRQOL) is an important oncologic end point for upper gastrointestinal malignancies. Unfortunately, till date, there is no published prospective data from India, comparing the HRQOL parameters between first-line chemotherapy regimens in advanced/metastatic gastric cancer. Materials and Methods: The present study aimed to compare the HRQOL of first-line systemic chemotherapy with epirubicin, cisplatin plus 5-FU (ECF) and docetaxel, cisplatin plus 5-FU (DCF) regimens in patients with locally advanced inoperable or metastatic gastric or gastro-esophageal junction adenocarcinoma. The secondary end points were overall response rate, progression-free survival (PFS), overall survival (OS), and toxicity profile. Results: Between December 2014 and December 2016, 65 patients were treated with ECF (n = 34) or DCF (n = 31) regimen. The baseline HRQOL scores were comparable between the two study groups, with the exception of significantly poor pain and sleep difficulties symptom score in the DCF group. After three cycles of treatment, both the groups showed improvements in most of the quality of life (QOL) parameters including global QOL score, compared with their baseline status. After six cycles of chemotherapy, the ECF group showed nonsignificant deterioration for most of the QOL parameters; but on the contrary, the DCF group maintained improved scores for most of the QOL parameters. The median survival until a definitive deterioration of global QOL score was significantly better in the DCF arm in comparison to the ECF arm (7.1 vs. 5.6 months, respectively, P = 0.000). The median OS was 9.2 months with ECF and 12.5 months with DCF regimen (P = 0.000), while median PFS was 5.7 and 7.4 months with ECF and DCF regimens, respectively (P = 0.002). Conclusions: This prospective study highlighted a better impact of DCF chemotherapy on the HRQOL of patients with advanced/metastatic gastric cancer and showed the importance of QOL assessments in clinical trials to complement the risk–benefit judgment.

Background: Epirubicin, cisplatin, and 5-FU (ECF) is one of the most commonly used first-line chemotherapy regimens in metastatic gastric cancer. However, due to protracted infusion schedule, need for special infusion pumps, and catheter-related complications, the practical utility and acceptability of standard ECF regimen are limited, particularly in resource-constrained settings including India. Materials and Methods: In the present study, we have used a more convenient modification of the standard ECF protocol (using 5 days intravenous infusion of 5-FU at a dose of 750 mg/m2/day, given over 6 h through a peripheral venous line), in Indian patients with metastatic gastric or gastroesophageal junction (GEJ) adenocarcinoma. The primary endpoint was overall survival (OS). The secondary endpoints were overall response rate (ORR), progression-free survival (PFS), and toxicity profile. Results: Between January 2014 and December 2017, 107 patients were assigned and treated with this modified ECF regimen. The median age was 52 years (range, 34–62); 66.3% were males and 36.5% of the patients had ≥ 3 metastatic disease site involvement at baseline. Dose reductions due to toxicity were required in 14.9% of the patients. The ORR was 32.7%; median PFS and OS were 5.9 months (95% confidence interval [CI]: 4.7–6.9) and 10.4 months (95% CI: 8.4–11.8), respectively. Both the hematological and nonhematological toxicities were manageable, and there was no toxicity-related death. The most frequent Grade 3–4 adverse events were neutropenia (18.7%), febrile neutropenia (13.1%), mucositis (5.6%), and diarrhea (5.6%). Conclusions: In the present study, the modified ECF regimen demonstrated significant efficacy with an acceptable toxicity profile in Indian patients with metastatic gastric and GEJ adenocarcinoma. The survival outcomes of this modified schedule were comparable with those of the standard ECF regimen, as reported earlier. Clearly, this modified and more convenient ECF protocol should be explored and validated through large prospective randomized trials.

Background: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the gastrointestinal tract. Historically, a poor prognosis for metastatic disease has been reported with systemic chemotherapy. Significant advances have been made in the last decade, since the introduction of different tyrosine kinase inhibitors (TKIs). Unfortunately, even though the TKIs have been used for a long time, there are very few published data of the experience of TKI therapy in metastatic GIST from India. Materials and Methods: Patients diagnosed with metastatic GIST from January 2005 to October 2016 at our center, who received first-line therapy with imatinib 400 mg/day, were reviewed retrospectively. Patients' profile, response to treatment, toxicity of TKI therapy, time to progression, and survival were evaluated. Results: Of the 44 metastatic GIST patients, 23 (52.2%) were males. Median age at diagnosis was 48 years. The most common presenting symptom was an abdominal pain (52%), followed by weight loss (23%). Most frequently affected metastatic site was liver (57%), followed by peritoneum (16%), and lungs (4.5%). Metastases to both liver and peritoneum were found in 10 patients (22.5%). All patients were initially treated with imatinib at a dose of 400 mg/day. Disease stabilization was documented in 21 cases (48%), and 13 patients (29%) achieved a partial response. TKI therapy was well-tolerated in most cases. Median progression-free survival (PFS) was 26 months, and estimated median survival was 48 months. Patients with lung metastases have a significantly inferior median PFS and overall survival, in comparison to patients with other metastatic sites (P < 0.05). Conclusions: Imatinib therapy was well tolerated and induced a sustained clinical benefit in more than half of the patients with metastatic GIST. Lung metastases seemed to be a poor prognostic factor in this patient population.

Background: Neuroendocrine carcinoma (NEC) is a rare tumor arising from the diffuse neuroendocrine system. Most of these present in the advanced stage and palliative chemotherapy remains the only option. The prognosis remains poor with the standard chemotherapy regimen of platinum and etoposide (EP) providing modest survival benefit. Methods: The study was done for 3 years at a tertiary cancer center in South India. Patients with a diagnosis of metastatic NEC were analyzed for clinical and pathological characteristics. The treatment outcomes and prognostic factors were evaluated using appropriate statistical test. Results: A total of 114 patients of metastatic NEC satisfied the inclusion criteria and were analyzed. Gastrointestinal including hepatobiliary tract (33%) was the most common site of primary disease followed by lung (26%), genitourinary (15%), head and neck (14%), and unknown primary (9%). On analysis of pattern of metastasis, liver (65%) was the most common site followed by bone (54%) and lung (42%). The median overall survival was 11 months with a statistically significant difference between pulmonary and extrapulmonary disease (8 vs. 13 months; P = 0.003). Ki67% value was strongly associated with prognosis (hazard ratio 0.517, 95% confidence interval; 0.318–0.840, P = 0.008) whereas age, sex, and lactate dehydrogenase level did not show any relation with survival. Conclusion: The outcome of advanced NEC with standard chemotherapy remains poor. Larger studies with other therapeutic and novel agents are warranted to improve the treatment outcomes.

Background: The incidence of lung cancer is rising in developing countries like India. Due to unaffordability among the low socioeconomic status (SES) patients, there is a significant delay in seeking appropriate medical treatment due to which a high proportion of patients present in an advanced/metastatic stage and the outcomes are poor. Objective: In this study, we studied the progression-free survival (PFS) and the pharmacoeconomic benefits with the cisplatin plus etoposide (EtoP) chemo regimen and compared it with the current generation chemo regimen. Materials and Methods: We performed a retrospective analysis of metastatic nonsmall cell lung cancer patients who received one or more cycles of platinum-based chemotherapy between 2011 and 2014. Results: Of the 304 patients, 56.6% of the patients were of the low SES. Of the low socioeconomic group patients, 67.45% and 31.4% received etoposide and paclitaxel platinum doublet combination regimen as first line, respectively. The mean PFS with the etoposide, paclitaxel, pemetrexed, and gemcitabine platinum-based doublet regimens were 9.35, 10, 10.76, and 9.83 months, respectively. Kaplan–Meier survival curve analysis showed a statistically significant initial survival with the first line EtoP cisplatin regimen for the initial 6 months of starting chemotherapy in comparison with the other regimens. Conclusions: This study showed a substantial pharmacoeconomic benefit with the cisplatin and etoposide chemo regimen in the lower socioeconomic group of patients. We believe that this is the first pharmacoeconomic study on metastatic non small cell lung treatment of great relevance to countries with limited resources.

Introduction: Ph+ AML is a well-recognized , but rare entity that poses challenges to clinicians to differentiate it from Chronic myeloid leukemia in blast crisis. Objective: Our aim was to study the clinical profile, treatment and outcomes of patients with Ph+ AML at our centre. Materials and Methods: Four cases of Ph+ AML entered in the hospital registry between 2013 and 2015 were identified and analysed. Results: The median age was 46 years with equal sex preponderance. The duration of presenting complaints was

Introduction: Plasmablastic lymphoma (PBL) is a rare lymphoma associated with immunosuppression. It is strongly associated with immunosuppression (human immunodeficiency virus [HIV]) and often occurs within the oral cavity. PBL is also seen in patients receiving immunosuppressive therapy; however, despite its predisposition for the immunocompromised patients, PBL has been diagnosed in immunocompetent patients. Aim: This study aims to prognostic factors and outcome of PBL in immunocompromised and in immunocompetent patients. Materials and Methods: We conducted a retrospective study at our institute from the year 2008 to 2015. Results: A total of 13 patients (8 males and 5 females) with PBL were identified. Eight patients (61.5%) had extraoral PBL (median age 30.2 years) and 5 patients (38.5%) had oral PBL (median age 44 years). Most common extraoral site was gastrointestinal tract. Eight (61.5%) out of 13 patients were HIV positive. More than 50% of patients had Ann Arbor Stage III or IV. All the cases were CD20 negative and CD138 positive. Seven out of 13 patients had Ki-67 more than 80%. Nine patients received cyclophosphamide, doxorubicin, vincristine, and prednisone chemotherapy. Three patients were on best supportive care due to poor performance status (PS). One patient received intensive chemotherapy with CODOX-M/IVAC. The median overall survival was 9 months in HIV-positive patients and 6 months in HIV-negative patients. The prognosis was worse in patients with Ki-67 of> 80%. Statistical Analysis: Survival curves were generated using the Kaplan–Meier method and analyzed using log-rank test and Fisher's t-test. Conclusion: The present study confirms that PBL in both HIV-positive and in HIV-negative patients has an overall unfavorable outcome. The most important prognostic factors are stage, ki-67, and the Eastern Cooperative Oncology Group PS of the patient at the time of presentation.

Introduction: Diffuse large B-cell lymphoma (DLBCL) is the most frequent non-Hodgkins lymphoma in the elderly. With the rising proportion of older persons in India, it is important to study current patterns and management of this disease, given that data in this regard are scarce in Indian settings. The aim of this study was to document the clinical features of DLBCL among elderly patients and their outcome over 7 years at a tertiary care oncology center. Materials and Methods: This was a retrospective records review of 119 DLBCL cases between January 2007 and January 2015 aged 60 years and above done at Kidwai Memorial Institute of Oncology, Bengaluru, Karnataka, India. Clinical staging was done according to Ann Arbor staging as modified by Cotswold's and International Prognostic Index (IPI) calculated. Results: The mean age was 69.54 years (±5.44) with male: female ratio of 1.52:1. B symptoms were seen in 33% of patients. Thirty-six percent of the patients had stage II disease. The advanced stage was seen in 12% and bulky disease in 9.5%. Bone marrow was involved in 12%. The most common extranodal site was the head and neck region. The distribution according to the IPI was as follows: Low risk 38 (31.93%), low-intermediate risk 53 (44.54%), high-intermediate risk 20 (16.80%), and high risk 8 (6.72%). Among 119 patients, 98 (64.7%) received treatment with either combination of rituximab, cyclophosphamide, adriamycin, vincristine, epirubicin, and prednisolone. Overall response rate was 63.26% with a complete response rate of 38.77%. The overall survival ranged from 2 to 123 months with the median being 9.5 months. Conclusion: In elderly, DLBCL is common in seventh decade and most of them present in an early stage and low IPI. The incorporation of rituximab to anthracycline based chemotherapy shows a significant improvement in survival in elderly DLBCL.

Background: Oral cavity cancer is a significant health problem in India. Majority of patients present with locally advanced disease requiring multimodality treatment. Compliance to recommended treatment is an important factor affecting outcome. Aims: The aim was to evaluate the outcome of locally advanced oral cavity cancer patients with regards to treatment adherence and to assess reasons of noncompliance. Materials and Methods: This was a prospective observational study. We included patients referred to Department of Medical Oncology for induction chemotherapy in view of locally advanced oral cavity cancer. Results: Only 15 (26%) patients completed planned treatment schedule. Their 1 year overall survival was 93%. The remaining 43 patients who received inadequate treatment had a dismal 21% 1 year overall survival. Illiteracy, poverty, long waiting list for surgery, prolonged delay for health scheme treatment plan approval and dissatisfaction with attitude of hospital staffs are major barriers related to effective treatment of these patients. Conclusions: A detailed discussion with patient and their relatives regarding recommended treatment, proper implementation of health schemes, increasing trained manpower to avoid long waiting list for surgery, provision of additional financial support for family member accompanying the patient and a sympathetic approach toward patients are needed to help these patients overcome the battle.

An oncologic emergency is defined as any acute, potentially life-threatening event, either directly or indirectly related to a patient′s cancer (ca) or its treatment. It requires rapid intervention to avoid death or severe permanent damage. Most oncologic emergencies can be classified as metabolic, hematologic, structural, or side effects from chemotherapy agents. Tumor lysis syndrome is a metabolic emergency that presents as severe electrolyte abnormalities. The condition is treated with aggressive hydration, allopurinol or urate oxidase to lower uric acid levels. Hypercalcemia of malignancy is treated with aggressive rehydration, furosemide, and intravenous (IV) bisphosphonates. Syndrome of inappropriate antidiuretic hormone should be suspected if a patient with ca presents with normovolemic hyponatremia. This metabolic condition usually is treated with fluid restriction and furosemide. Febrile neutropenia is a hematologic emergency that usually requires inpatient therapy with broad-spectrum antibiotics, although outpatient therapy may be appropriate for low-risk patients. Hyperviscosity syndrome usually is associated with Waldenstrφm′s macroglobulinemia, which is treated with plasmapheresis and chemotherapy. Structural oncologic emergencies are caused by direct compression of surrounding structures or by metastatic disease. Superior vena cava syndrome is the most common structural oncological emergency. Treatment options include chemotherapy, radiation, and IV stenting. Epidural spinal cord compression can be treated with dexamethasone, radiation, or surgery. Malignant pericardial effusion, which often is undiagnosed in ca patients, can be treated with pericardiocentesis or a pericardial window procedure.

We report a case of a 17-year-old male with precursor B acute lymphoblastic leukemia who presented with erythematous palpable rash over all extremities and trunk following initiation of linezolid for febrile neutropenia. This rash resolved on discontinuation of linezolid. We report this case with emphasis on review of the literature to highlight this unusual adverse effect and make clinicians aware and create a high index of suspicion in such a situation.

Plasma cell leukemia (PCL) is a rare entity. There are two presentations of PCL, primary or secondary. The primary or de novo form of PCL presents with an acute and rapidly progressive leukemic phase. This form occurs when the patient has no pre-existing multiple myeloma (MM). The secondary form is the most advanced form of MM. The PCL is a rare disorder representing 1‒2% of the diagnosed cases of MM. Median age at presentation is usually above 50 years. The monoclonal protein in patients with PCL may be IgG (50%), IgA (15%), or in rare cases IgD or IgE (6%). We report a case of IgA primary PCL that is very rare. Patient was started on combination therapy with vincristine, adriamycin, and dexamethasone. There was poor response and patient died three months after diagnosis.