Epidemiologic and Clinical Patterns of Malignant Lymphoma in Qatar 2013-2017: A population-based Cohort Study.

BACKGROUND: Lymphoma, encompassing common non-Hodgkin lymphoma (NHL) and less common Hodgkin lymphoma (HL), represents significant hematological malignancies. Advancements in treatment modalities have reshaped survival rates, particularly in NHL. This complexity results in varying outcomes, some requiring extended observation periods and multiple chemotherapy treatments. OBJECTIVE: The primary objective is to explore and compare the overall survival (OS) of HL and NHL at 1, 3, and 5-year follow-ups among adult lymphoma patients in Qatar during January 2013 - December 2017. Further objectives encompass comparing the most prevalent histological types, clinical and epidemiological traits of HL and NHL, as well as secondary aims of assessing clinical features, treatment, response, disease-free survival, and overall survival. METHODS: A retrospective, descriptive study of consecutive cases was conducted at Qatar's NCCCR between 2013 and 2017. Inclusion criteria involved patients ≥18 years old, of any gender and clinical stage at diagnosis, who received chemotherapy and had known outcomes. Descriptive statistics were applied, and survival analysis utilized Kaplan-Meier curves. STATA version 13.0 facilitated data analysis. RESULTS: Between 2013-2017, 414 individuals in Qatar were diagnosed with lymphoma. The median age at diagnosis was 49 years (IQR 36-95 years; p<0.001) across all patients. Males exhibited a higher likelihood of developing HL and NHL, comprising 74% and 70% of cases respectively, though this difference was statistically insignificant (p=0.45). Among NHL-B subtypes, mature B-cell neoplasms (60%) predominated, while Lymphocyte-rich subtype (49%) was prominent in HL cases. With a median follow-up of 17.3 months, OS rates at 1, 3, and 5 years were 99%, 82%, and 64% respectively for all lymphoma patients. Subtype stratification revealed trends in 3-year follow-up OS (94% versus 82%) for HL and NHL, with 5-year OS of 67% and 60% respectively. HL demonstrated higher OS throughout the study period compared to NHL (p<0.001), though median OS remained unreached. CONCLUSIONS: Diffuse large B-cell lymphoma emerged as the most prevalent subtype among lymphomas in Qatar. Generally, HL exhibited superior survival rates, at 67% compared to 60% for NHL. Minor deflation in survival rates, particularly for HL, might be attributed to Qatar's immigration patterns.

Carfilzomib-induced life-threatening lung injury in refractory multiple myeloma.

INTRODUCTION: Carfilzomib is a second-generation selective proteasome inhibitor that is commonly used in the treatment of relapsed or refractory multiple myeloma. Carfilzomib is associated with respiratory side effects, such as cough, dyspnea, and upper respiratory tract infection. However, severe pulmonary toxicity is rare and is only reported in a few case reports. CASE REPORT: Here, we present a case of a 65-year-old male with refractory multiple myeloma who developed a life-threatening lung injury during his third cycle of carfilzomib. The patient presented with a decreased level of consciousness and was found to have Type I respiratory failure. He was admitted to the intensive care unit, where he was intubated. Blood cultures and viral panel were negative. The patient received a prolonged course of antibiotics with 2 days of hydrocortisone. MANAGEMENT AND OUTCOMES: After discharge, repeated myeloma workup showed disease progression and carfilzomib was reintroduced. The next day, he presented with fever, vomiting, and hypoxia. Chest x-ray showed congestive lung changes with patchy airspace opacities. Repeated echocardiography showed normal ejection fraction with moderate pulmonary hypertension (RVSP 46 mm Hg). The patient was transferred again to the ICU and kept on continuous positive airway pressure. Antibiotics were started, and blood cultures and respiratory viral panels were negative for any infectious organism. The patient improved in terms of inflammatory markers and oxygen requirements. Treatment with carfilzomib was stopped permanently. DISCUSSION: Pulmonary toxicity associated with carfilzomib in patients with multiple myeloma can be potentially life-threatening. The mechanism with which carfilzomib induces lung-related AEs is still not fully understood. In our patient, carfilzomib-induced lung injury was evident after rechallenging the patient with carfilzomib, in the radiographic x-ray changes and the new onset moderate pulmonary hypertension. Healthcare providers should be encouraged to report rare adverse events in order to identify the risk factors that can predispose patients to the development of these adverse events.

Dilemma of Thrombolysis in a patient with high-risk Pulmonary Embolism with severe Thrombocytopenia: A case report.

BACKGROUND: Managing a high-risk pulmonary embolism (PE) in a critically ill patient with severe thrombocytopenia can present a challenging dilemma. There is a high risk of fatal bleeding due to anticoagulation in high-risk PE with thrombocytopenia; therefore, risks and benefits are balanced while dealing with such a critical scenario. CASE REPORT: We present a case of a female patient with thrombocytopenia who was admitted for management of lymphoma. Her hospital course was complicated by high-risk PE, leading to acute respiratory failure and hypotension, necessitating urgent transfer to the medical intensive care unit. She was intubated and placed on mechanical ventilation. Multiple cardiac arrests occurred due to compromised cardiac output from a severely dilated right ventricle on bedside transthoracic echocardiography. As a last resort to save her life in this critical state and severe thrombocytopenia, she was given a half bolus dose of the recommended drug, i.e., 50mg IV of Alteplase. Subsequently, she stabilized and was extubated without any further complications. DISCUSSION: High-risk PE needs prompt management with anticoagulation to avoid fatal outcomes. However, on the other hand, anticoagulation carries a high risk of bleeding, especially in patients with thrombocytopenia. These challenges prompt a modern perspective in situations where clear guidelines are absent. CONCLUSION: We aim to discuss our contemporary clinical practice in managing such a complex case and highlight the need for further studies.

Severe Acute Respiratory Syndrome Coronavirus 2 Omicron Variant in Patients with Philadelphia-Negative Myeloproliferative Neoplasm: A Single Center Experience.

INTRODUCTION: Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) is the viral agent responsible for the coronavirus disease of 2019. The disease is primarily a respiratory illness; however, multisystem involvement is not uncommon. The infection is reported to be more severe in patients with multiple comorbidities and immunocompromised patients. Patients with hematological malignancies are immunocompromised and prone to develop severe SARS-CoV-2 infection. The SARS-CoV-2 had developed several mutations that resulted in different strains with different virulence and different degree of protection by vaccination or prior infection. The Omicron variant is reported to cause mild illness; however, the effect on patients with hematological malignancies like myeloproliferative neoplasms (MPNs) is not clear. We present patients with MPNs who had infection with the Omicron variant of the SARS-CoV-2 and their outcomes. METHODS: Retrospective data from the National Center for Cancer Care and Research records from December 20, 2021, to January 30, 2022. Participants were adults over the age of 18 years with Omicron infection who had been diagnosed with Philadelphia-negative MPNs, essential thrombocythemia, polycythemia vera (PV), and primary myelofibrosis according to the 2008/2016 WHO classification for MPN. RESULTS: Twenty-two patients with Philadelphia-negative MPN had Omicron infection. All patients had a mild disease according to the WHO classification of COVID-19 severity. Most of the patients had medical comorbidities, with hypertension being the most common comorbidity. However, only one patient with PV required hospitalization. DISCUSSION/CONCLUSIONS: In patients with Philadelphia-negative MPN, the Omicron variant of SARS-CoV-2 usually results in mild infection.

Frequency of Acute Kidney Injury in Patient Receiving Piperacillin - Tazobactam: A Hospital-based Study from Qatar.

BACKGROUND: several studies have been reported piperacillin-tazobactam (TAZ / PIPC)-associated AKI with various frequencies. The aim of this study was to determine the frequency of TAZ/PIPC- associated AKI among our patients and to identify the risk factors for this clinical entity. METHODS: this retrospective cross-sectional study was conducted at Hamad General Hospital; it involved adult patients who were admitted from January 2017 to December 2017. RESULTS: we involved 917 patients, of whom 635 (69.25%) were males and 282 (30.75%) were females. The mean age of the patients was 52 (SD 19) years, and 98 (10.7%) patients were diagnosed with AKI. The patients with AKI were significantly older than without AKI [59.71 (SD 19.79) versus 51.06 (SD 18.67); P <0.001]. After TAZ/PIPC initiation, the mean creatinine level in the AKI group was higher than the mean creatinine level in the non-AKI group, [158.91 (SD 81.93) versus 66.78 (SD 21.42); P<001]. The mean time of onset of AKI after PIPC/TAZ initiation was 4.46 (SD 3.20) (1-12 days). AKI was significantly associated with low mean serum albumin (P<0.001), high mean fasting blood glucose (P<0.001), coronary artery diseases (P<0.001), heart failure (P<0.001), liver diseases (P=0.047), diabetes mellitus (P=0.021) and hypertension (P<0.001). The in-hospital mortality was significantly higher in the AKI group [38.78% versus 5.13% in the non-AKI group; P<0.001], and only advanced age and heart failure were found as independent risk factors for TAZ/PIPC-associated AKI. CONCLUSION: TAZ/PIPC was significantly associated with AKI. Advanced age and heart failure were identified as independent risk factors for TAZ/PIPC-associated AKI.

Association of vitamin B12 deficiency with metformin use in patients with type 2 diabetes treated in the largest tertiary care hospital in Qatar.

BACKGROUND: Data on the effect of metformin on serum vitamin B12 (VitB12) level in patients with type 2 diabetes mellitus (T2DM) in Qatar are limited; therefore, we aimed to assess the prevalence of VitB12 deficiency and its related factors among patients with tbl2DM treated with metformin at Hamad General Hospital in Doha, Qatar, from January 1, 2017, to December 31, 2017. METHODS: This cross-sectional analytical study involved patients with tbl2DM aged ≥ 18 years who used metformin for at least 3 months. The serum VitB12 was quantified on a chemiluminescent enzyme immunoassay analyzer using Cobas e 801 module, Roche, and VitB12 deficiency was defined as serum VitB12 level of ≤ 145 pmol/L. All data were obtained from the patients' electronic medical records. RESULTS: The study recruited 3124 eligible patients with tbl2DM. The overall prevalence of metformin-associated VitB12 deficiency was 30.7% [95% confidence of interval, 0.290-0.323]. A significant difference exists in the median VitB12 levels between the VitB12-normal and VitB12-deficient groups [129 vs. 286; p < 0.001]. Compared with the VitB12-normal group, the VitB12-deficient group had higher mean body mass index (BMI) (p < 0.001) and consumed higher doses of metformin (p = 0.001). They also more often used sulfonylurea (p = 0.004), dipeptidyl peptidase-4 inhibitor (p < 0.001), thiazolidinediones (p < 0.001), glucagon-like peptide 1 [GLP-1] receptor agonists (p < 0.001), alpha-glucosidase inhibitor (p < 0.001), and H2 blocker/proton pump inhibitors [PPI] (p < 0.001) than the VitB12-normal group. Moreover, the VitB12-normal group consumed more calcium supplements (p < 0.001) than the VitB12-deficient group. In the multivariate analysis, independent risk factors for metformin-associated VitB12 deficiency in patients with tbl2DM include high daily dose of metformin >2000 mg, male gender, high BMI, smoking, sulfonylurea, dipeptidyl peptidase-4 inhibitor, H2 blockers/PPI, low fasting blood glucose, and low hemoglobin. CONCLUSION: This study showed a high prevalence of VitB12 deficiency in patients with tbl2DM taking metformin and a significant negative correlation between the daily dose of metformin and serum VitB12 level. Therefore, regular screening for serum VitB12 is necessary in patients with tbl2DM on metformin treatment, especially those who have the abovementioned risk factors.

Safety and Efficacy of Asciminib in Chronic Myeloid Leukemia Patient with Chronic Kidney Diseases: A Case Report.

Introduction: Chronic myeloid leukemia (CML) is characterized by the presence of the BCR::ABL1 fusion gene, and the advent of tyrosine kinase inhibitors (TKIs) has revolutionized its therapeutic landscape. Asciminib, a STAMP inhibitor, emerges as a promising option for patients unresponsive or intolerant to multiple conventional TKIs. However, the safety and efficacy of asciminib in individuals with chronic kidney disease remain understudied. Case Presentation: In this report, we detail the case of a 62-year-old patient with CML and stage 3 chronic kidney disease, who faced intolerance to traditional TKIs primarily due to fluid retention. The transition to asciminib therapy resulted in a profound molecular response and did not exacerbate renal function, effectively addressing the fluid retention issue. Conclusion: This case highlights the potential of asciminib as a viable therapeutic alternative for CML patients with chronic kidney disease, particularly those intolerant to standard TKIs. Further research is warranted to establish the broader safety and efficacy profile of asciminib in this patient population.

Luspatercept's use in a patient with transfusion-dependent beta-thalassemia and intrathoracic extramedullary hematopoiesis (EMH).

Key Clinical Message: This case report and literature review examine the use of a relatively novel agent in a transfusion-dependent beta-thalassemia patient with extramedullary hematopoiesis (EMH). It examines the benefits and risks associated with its use and reviews the available literature while highlighting the drug's results in our patient with a higher risk profile. Abstract: Beta thalassemia can be complicated by EMH, which causes different symptoms based on location and size. Luspatercept is a new agent approved for transfusion-dependent thalassemia and Non-transfusion-dependent thalassemia (NTDT). Still, its use in patients with EMH was not well studied, and literature showed an increased risk of EMH expansion or development of new masses after its use. We discuss, in this case, the results of luspatercept treatment in a patient with transfusion-dependent thalassemia who is considered high risk for its use due to the patient's specific characteristics (history of symptomatic intrathoracic EMH, previous splenectomy, refusal to use antithrombotic medications). While also highlighting the benefits of using luspatercept regarding decreasing the iron overload and improving hemoglobin levels and examining how it was used safely to manage a transfusion-dependent thalassemia patient with an extramedullary hematopoiesis mass with no adverse events of note.

Potential efficacy of crizanlizumab in treating priapism in sickle cell disease: A case report.

This report documents the treatment of a 41-year-old male with sickle cell disease (SCD) and repeated stuttering priapism using crizanlizumab, which alleviated the priapism but induced a significant vaso-occlusive crisis during the second infusion. Encouragingly, no subsequent vaso-occlusive crises occurred. However, the potential for infusion-related adverse events warrants close supervision. Further research is necessary to explore its full benefits on priapism management.

Primary Splenic Diffuse Large B-Cell Lymphoma: A Case Report and Literature Review of a Rare Condition.

Introduction: Primary splenic lymphoma is a rare lymphoproliferative disorder that involves the spleen, exhibits diverse clinical presentations, and lacks a clear consensus in terms of management strategies. Case Presentation: We present the case of a 52-year-old patient with a complex medical history marked by multiple chronic medical conditions. The patient was diagnosed with primary splenic lymphoma, specifically the diffuse large B-cell subtype. Treatment for our patient involved a shortened course of chemotherapy (4 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone [R-CHOP] followed by two doses of rituximab) due to issues related to compliance and treatment-related complications. This was followed by consolidative radiotherapy without resorting to splenectomy. Conclusion: Remarkably, despite using a shortened course of R-CHOP, the patient achieved complete resolution, and a positron emission tomography scan conducted at the end of the 6-month posttreatment period confirmed sustained complete remission.

Obstructive Sleep Apnea Does Not Exclude Polycythemia Vera: A Case Report.

Introduction: Polycythemia vera (PV) is one of the myeloproliferative neoplasms (MPN) diagnosed by World Health Organization (WHO) criteria 2016, which requires the presence of 3 major criteria: high hemoglobin/hematocrit, bone marrow findings, and Janus Kinase 2 (JAK2) mutation or two major and one minor criteria, including erythropoietin (EPO) level. However, in clinical practice, difficulties in diagnosis can arise as it may be masked by secondary causes for erythrocytosis such as smoking or obstructive sleep apnea (OSA). Case Presentation: Here, we report a 55-year-old gentleman, morbidly obese with OSA on home continuous positive airway pressure (CPAP) machine, who was incidentally found to have polycythemia. Further evaluation confirmed the diagnosis of PV. Conclusion: PV can be masked by the assumption of secondary polycythemia based on history. This underscores the importance of screening such cohort through JAK2 and EPO testing to avoid missing PV.

Cost-effectiveness of l-glutamine versus crizanlizumab for adults with sickle cell disease: model focused on reducing pain episode costs from Qatar's healthcare perspective.

Objectives: Treatment options for preventing vaso-occlusive crises among sickle cell disease patients are on the rise, especially if hydroxyurea treatment has failed. This economic analysis is conducted to assess the comparative clinical effectiveness, safety, and acquisition cost of l-glutamine and crizanlizumab for older adolescents and adults (⩾16 years old) with sickle cell disease in Qatar, with an emphasis on treatment costs and acute pain crises. Methods: We conduct a decision-tree model, where we compare the clinical and economic outcomes of two novel Food and drug administration (FDA)-approved medications which are available in Qatar; l-glutamine and crizanlizumab over a time horizon of 1 year in a hypothetical cohort of adult sickle cell disease patients from a Qatar healthcare perspective. The main outcome is incremental cost per sickle cell disease-related acute pain crises averted. Model clinical parameters were derived from individual drug randomized trials, published literature, whereas cost parameters from Qatar healthcare payer system (2020-2021). A sensitivity analysis was carried out, and the study results were robust around model inputs. Costs were converted to 2020 US dollars. Results: Study results showed that both treatment modalities' costs were the main driver of this analysis, with an average annual cost of the treatments per patient being $189,014 for crizanlizumab (5 mg/kg), $143,798 for crizanlizumab (2.5 mg/kg), and $74,323 for l-glutamine. The probability of no first-time sickle cell disease-related vaso-occlusive crises averted was 0.001/year for glutamine, 0.26/year for crizanlizumab (5 mg/kg), and 0.34/year for crizanlizumab (2.5 mg/kg). Lower dose crizanlizumab (2.5 mg/kg) dominated the higher one (5 mg/kg). The incremental cost-effectiveness ratio of crizanlizumab (2.5 mg/kg), when compared to l-glutamine was $81,265 per sickle cell disease-related vaso-occlusive crises averted. When comparing crizanlizumab (5 mg/kg) and l-glutamine, crizanlizumab (5 mg/kg) showed higher efficacy, yet the crizanlizumab incremental cost-effectiveness ratio was at $459,620 than l-glutamine. Conclusions: Crizanlizumab (2.5 mg/kg) may be a cost-effective intervention, yet it is not the approved dose for preventing vaso-occlusive crises in adolescents and adults with sickle cell disease. Crizanlizumab (5 mg/kg) was more cost-effective than the approved l-glutamine per sickle cell disease vaso-occlusive crisis prevented. Of note, we primarily focused on modeling acute vaso-occlusive pain, which limited our ability to consider other key outcomes in sickle cell disease.

The Contemporary Role of Hematopoietic Stem Cell Transplantation in the Management of Chronic Myeloid Leukemia: Is It the Same in All Settings?

Hematopoietic stem cell transplantation (HSCT) for chronic myeloid leukemia (CML) patients has transitioned from the standard of care to a treatment option limited to those with unsatisfactory tyrosine kinase inhibitor (TKI) responses and advanced disease stages. In recent years, the threshold for undergoing HSCT has increased. Most CML patients now have life expectancies comparable to the general population, and therefore, the goal of therapy is shifting toward achieving treatment-free remission (TFR). While TKI discontinuation trials in CML show potential for achieving TFR, relapse risk is high, affirming allogeneic HSCT as the sole curative treatment. HSCT should be incorporated into treatment algorithms from the time of diagnosis and, in some patients, evaluated as soon as possible. In this review, we will look at some of the recent advances in HSCT, as well as its indication in the era of aiming for TFR in the presence of TKIs in CML.

The characteristics of CALR mutations in myeloproliferative neoplasms: a clinical experience from a tertiary care center in Qatar and a literature review.

BACKGROUND: Myeloproliferative neoplasms (MPNs) are hematological disorders characterized by abnormal production of myeloid cells due to genetic mutations. Since 2013, researchers have identified somatic mutations in the Calreticulin (CALR) gene, primarily insertions or deletions, in two Philadelphia chromosome-negative MPNs; essential thrombocytosis (ET) and primary myelofibrosis (PMF), and occasionally in chronic myelomonocytic leukemia (CMML). This study aims to identify the various types of CALR mutations and their impact on CALR-positive MPN patients' clinical manifestations and outcomes. METHODS: A single-center retrospective study was conducted. The data was collected from pre-existing records. The study was carried out on Philadelphia-negative MPN patients who were being followed up on at the NCCCR (National Center for Cancer Care and Research) to assess the clinical manifestation and outcome of disease treatment. All patients included, were followed in our center between January 1, 2008, and November 20, 2021. RESULTS: A total of 50 patients with CALR-positive MPN were reviewed with a median follow-up of three years (1-11). This cohort included 31 (62%) patients with ET, 10 (20%) patients with PMF, and 9 (18%) patients with prefibrotic myelofibrosis (pre-MF). The study involved 38 (76%) male and 12 (24%) female patients. There were 16 (32%) patients diagnosed before the age of 40, 24 (48%) patients diagnosed between the ages of 40 and 60; and 10 (20%) patients diagnosed after the age of 60. Molecular analysis showed 24 (48%) patients with CALR type 1, 21 (42%) patients with CALR type 2, and 5 (10%) patients with none Type 1, none Type 2 CALR mutations. Two patients have double mutations; 1(2%) with none Type 1, none Type 2 CALR and JAK2 mutations, and 1(2%) with CALR type 1 and MPL mutations. The thrombotic events were 3 (6%) venous thromboembolisms, 3 (6%) abdominal veins thromboses, 2 (4%) strokes, and 4 (8%) ischemic cardiac events. Only 4 (8%) patients progressed to Myelofibrosis and were carrying CALR 1 mutations, and 1 (2%) patient progressed to AML with CALR 2 mutation. CONCLUSION: The data shows a significant rise in CALR-positive MPN diagnoses in younger people, emphasizing the need for a better assessment tool to improve disease management and reduce complications.

Erythema nodosum following treatment with dasatinib plus chemotherapy in a patient with myeloid blast phase of chronic myeloid leukemia.

Erythema nodosum (EN) is a type of panniculitis occurring due to various conditions. It can be associated with certain malignancies or manifest as a side effect of drugs. This article presents a unique case of EN in a patient with chronic myeloid leukemia (CML-blast phase) following dasatinib and chemotherapy. Timely recognition and appropriate management are crucial to alleviate symptoms and consider potential drug-induced etiology.

Refractory cold agglutinin disease successfully treated with daratumumab. A case report and review of literature.

BACKGROUND: Cold agglutinin disease (CAD) is immune-mediated hemolytic anemia. The disease is caused by cold reactive autoantibodies that induce hemolysis through the activation of the complement pathway. Most patients with CAD are elderly, and half may have refractory CAD that may not respond to the first-line treatment option (i.e. rituximab). Some cases are refractory to multiple lines of therapy, including chemotherapeutic agents, which might be toxic, especially for elderly patients. Daratumumab is a human monoclonal antibody targeting CD 38 glycoprotein, a transmembrane protein highly expressed in lymphoid and plasma cells. Daratumumab is currently approved for treating multiple myeloma and is used mainly as a combination therapy with other agents. CASE PRESENTATION: Our patient is a 69-year-old female diagnosed with CAD after presenting with severe anemia and significant circulatory symptoms. Rituximab was not effective in controlling her disease, and she refused other available chemotherapeutic agents due to their side effects profile. We used daratumumab combined with erythropoietin, which led to a dramatic response measured by stabilizing her hemoglobin levels and transfusion independence. CONCLUSION: Our case is the third reported case of refractory CAD successfully treated with daratumumab, which suggests that daratumumab might be a potential agent for the treatment and control of refractory Cold Agglutinin Disease.

Sickle cell leg ulcer successfully managed by hyperbaric oxygen: a case report.

Sickle cell leg ulcers (SCLUs) are usually chronic, painful, and devastating complications of sickle cell disease. Skin vaso-occlusion with compromised blood flow, chronic inflammation, and endothelial dysfunction is thought to be the underlying mechanism. It is usually slow to heal, and it may become chronic and superinfected. The management of SCLUs is usually challenging and requires a multidisciplinary team. Multiple systemic and local therapies have been tried in SCLU treatment. However, the outcome is variable: currently, there are no official recommendations for the best effective treatment. Herein, we report a 34-year-old male patient with non-transfusion-dependent sickle cell disease who was suffering from a chronic left ankle ulcer and was successfully managed with hyperbaric oxygen therapy, resulting in a complete resolution of this devastating complication.

Delayed Diagnosis of Indolent Systemic Mastocytosis as the Cause of Unexplained Skin Rash: A Case Report.

Mastocytosis is a heterogeneous group of disorders in which mast cells exhibit clonal proliferation that infiltrates one or more organs. In cutaneous mastocytosis, the mast cells infiltrate the skin only, whereas systemic mastocytosis is diagnosed when at least one extra-cutaneous site is involved, with or without the skin being affected. Given the rarity of mastocytosis and the fact that skin rash can be a manifestation of different conditions and many clinicians are not familiar with this disorder, an accurate diagnosis may be delayed. We report a delayed diagnosis of indolent systemic mastocytosis in a 40-year-old gentleman who had been complaining of an unexplained skin rash for 6 years.

Langerhans Cell Histiocytosis with Good Response to Low-Dose Imatinib: Case Report and Literature Review.

Langerhans cell histiocytosis (LCH) is a rare neoplastic disease characterized by infiltration of histiocytes and dendritic cells into body organs. While treatment is better established in pediatrics, there is still no consensus on therapy in the adult population. Imatinib has shown promising results in some case reports and a small clinical trial. We present here a fifty-nine-year-old patient with LCH in the lung, liver, and bone who responded well to an imatinib dose of 100 mg daily. Her symptoms improved within 3 months of treatment, and subsequent positron emission tomography-computed tomography (PET/CT) showed resolution of 18F-fluorodeoxyglucose (FDG)-avid lesions.

Spontaneous Remission in a Patient with Chronic Myeloid Leukemia: A Case Report.

Chronic myeloid leukemia (CML) is a myeloproliferative neoplasm in which granulocytic cells are the main proliferative component. At diagnosis, more than 90% of CML cases have the characteristic Philadelphia chromosome, containing the BCR::ABL1 fusion gene. The natural history of untreated CML is an initial indolent chronic phase which will be followed by an accelerated phase, blast phase, or both. Tyrosine kinase inhibitors (TKIs) have dramatically altered the natural history of CML. TKI discontinuation with the goal of treatment-free remission is currently part of current management recommendations. However, spontaneous remission without receiving any treatment is extraordinarily rare in CML patients. Herein, we report a 56-year-old male who presented with leukocytosis and was diagnosed as a case of CML in the chronic phase; however, treatment with TKIs was not initiated due to spontaneous hematological as well as molecular remission.