Treatment outcomes between cyclosporin and chemotherapy in adult subcutaneous panniculitis-like T-cell lymphoma: a report from nation-wide Thai lymphoma study group registry.

Subcutaneous panniculitis-like T-cell lymphoma (SPTCL) is a rare subtype of T-cell lymphomas with a characteristic feature of subcutaneous nodules associated with hemophagocytic lymphohistiocytosis (HLH). Treatment options for SPTCL are mainly chemotherapy (CMT) or immunosuppressive agents with selection currently dependent on physician decisions. Outcomes between the 2 treatment remedies have not yet been comprehensively compared. This study aimed to compare complete remission (CR) rates between SPTCL patients receiving cyclosporin (CSA)-based regimen (CSA +/- steroid) and CMT. The 5-year overall survival (OS) and 5-year progression free survival (PFS) were also analyzed. Clinical data from patients with SPTCL were drawn from the Thai Lymphoma Study Group registry who were newly diagnosed between 2007 and 2023. A total of 93 patients were selected with 45 cases having received CSA-based regimen and 48 cases having received CMT. There were more patients with limited stage at skin in the CSA group (63.8% vs. 36.2%, p = 0.003), while more patients with hepato- and/or splenomegaly were found in the CMT group (56.2% vs. 24.5%; p = 0.002). Germline HAVCR2 mutations were detected in 26/33 (78.8%) cases. The CR rate was significantly higher in patients treated with CSA (87% vs. 58.3%; OR = 6.5 [95%CI, 2.7-15.3]; p = 0.002). At a median follow-up of 87.8 months (range 0-185), the 5-year OS (98% vs. 87%, p = 0.19) and PFS (72.4% vs. 69.2%, p = 0.19) showed a trend favoring patients treated with CSA. Based on our study, CSA-based regimens are the preferred first-line treatment remedy for newly diagnosed SPTCL, especially in patients with limited cutaneous involvement.

Adding MYC/BCL2 double expression to NCCN-IPI may not improve prognostic value to an acceptable level.

BACKGROUND: MYC/BCL2 double expression (DE) is associated with poor prognosis in patients with diffuse large B-cell lymphoma (DLBCL) receiving rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisolone (R-CHOP). This study aimed to determine whether the addition of DE to the National Comprehensive Cancer Network Internal Prognostic Index (NCCN-IPI) could improve the prediction of disease progression in patients with DLBCL treated with R-CHOP. METHODS: This confirmatory prognostic factor study retrospectively recruited patients with newly diagnosed DLBCL between January 1, 2014, and January 31, 2018, at Ramathibodi Hospital (RA) and Thammasat University Hospital (TU). The follow-up period ended on July 1, 2022. Tumors expressing MYC ≥ 40% and BCL2 ≥ 50% were classified as DE. We calculated the hazard ratios (HR) for progression-free survival (PFS) from the date of diagnosis to refractory disease, relapse, or death. Discrimination of the 5-year prediction was based on Cox models using Harrell's concordance index (c-index). RESULTS: A total of 111 patients had DE (39%), NCCN-IPI (8%), and disease progression (46%). The NCCN-IPI adjusted HR of DE was 1.6 (95% confidence interval [CI]: 0.9-2.8; P = 0.117). The baseline NCCN-IPI c-index was 0.63. Adding DE to the NCCN-IPI slightly increased Harrell's concordance index (c-index) to 0.66 (P = 0.119). CONCLUSIONS: Adding DE to the NCCN-IPI may not improve the prognostic value to an acceptable level in resource-limited settings. Multiple independent confirmatory studies from a large cohort of lymphoma registries have provided additional evidence for the clinical utility of DE.

Endothelial activation and stress index as a prognostic factor of diffuse large B-cell lymphoma: the report from the nationwide multi-center Thai Lymphoma Study Group.

Several prognostic models have been introduced to predict outcomes of patients with diffuse large B-cell lymphoma (DLBCL). Endothelial activation and stress index (EASIX) is a surrogate of endothelial dysfunction which has been shown to predict outcomes of patients with various hematologic malignancies. However, the prognostic implication of EASIX for DLBCL is limited and warrants exploration. We conducted a retrospective study enrolling adult DLBCL patients including a discovery cohort from the single-centered university hospital database and a validation cohort from the independent nationwide multi-center registry. EASIX scores were calculated using creatinine, lactate dehydrogenase, and platelet levels. The receiver operating characteristic curve analysis was used to determine optimal cutoff. Statistical analysis explored the impact of EASIX on survival outcomes. A total of 323 patients were included in the discovery cohort. The optimal EASIX cutoff was 1.07 stratifying patients into low (53.9%) and high EASIX (46.1%) groups. Patients with high EASIX had worse 2-year progression-free survival (PFS) (53.4% vs. 81.5%, p<0.001) and overall survival (OS) (64.4% vs. 88.7%, p<0.001) than patients with low EASIX. Multivariate analysis revealed that older age, bulky disease, impaired performance status, and high EASIX were associated with an unfavorable OS. In the validation cohort of 499 patients, the optimal EASIX cutoff was 1.04. Similar to the discovery cohort, high EASIX score was associated with high-risk diseases, worse PFS, and inferior OS. In conclusion, EASIX score was significantly associated with survival outcomes and may be used as a simple prognostic tool to better risk-classify DLBCL.

Outcomes of polatuzumab vedotin-containing regimens in real-world setting of relapsed and or refractory diffuse large B-cell lymphoma patients: a matched-control analysis from the Thai Lymphoma Study Group (TLSG).

Relapsed/refractory diffuse large B-cell lymphoma (R/R DLBCL) is a challenging condition to treat, and there is an unmet clinical need for effective therapies. Recently, polatuzumab vedotin (Pola), an anti-CD79b antibody-drug-conjugate (ADC), combined with bendamustine-rituximab (BR), has been approved for R/R DLBCL patients. However, real-world data on Pola-based regimens in R/R DLBCL patients, especially in Thailand, are limited. This study aimed to evaluate the efficacy and safety of Pola-based salvage treatment in R/R DLBCL patients in Thailand. Thirty-five patients who received Pola-based treatment were included in the study, and their data were compared to 180 matched patients who received non-Pola-based therapy. The overall response rate (ORR) in the Pola group was 62.8%, with complete remission and partial remission rates of 17.1% and 45.7%, respectively. The median progression-free survival (PFS) and overall survival (OS) were 10.6 months and 12.8 months, respectively. The study found a significantly higher ORR in Pola-based salvage treatments compared to non-Pola-based therapy (62.8% vs. 33.3%). The survival outcomes were also significantly superior in the Pola group, with longer median PFS and OS than the control group. Grades 3-4 adverse events (AEs) were mainly hematological, and they were tolerable. In conclusion, this study provides real-world evidence of the efficacy and safety of Pola-based salvage treatment in R/R DLBCL patients in Thailand. The results of this study are promising and suggest that Pola-based salvage treatment could be a viable option for R/R DLBCL patients who have limited treatment options.

External validation and comparison of IPI, R-IPI, and NCCN-IPI in diffuse large B-cell lymphoma patients treated with R-CHOP to predict 2-year progression-free survival.

BACKGROUND: Diffuse large B-cell lymphoma (DLBCL) is the most common aggressive lymphoma. The standard first-line therapy for DLBCL consists of rituximab cyclophosphamide doxorubicin vincristine and prednisone (R-CHOP). About 50-70% of patients may be cured by R-CHOP. There was no data on external validation and comparison of the international prognostic index, revised-IPI (R-IPI), and enhanced-IPI (NCCN-IPI) to predict treatment outcomes in the middle-income country with a resourced-limited setting. OBJECTIVES: We aimed to externally validate and compare IPI, R-IPI, and NCCN-IPI in predicting 2-year progression-free survival (2-y PFS) of newly diagnosed DLBCL patients treated with R-CHOP. METHODS: This ambispective observational study recruited consecutive patients diagnosed between 1 January 2014 and 30 June 2020, with the last follow-up on 1 July 2022 from Thammasat University Hospital and Ramathibodi Hospital. We assessed discrimination by Harrell's concordance index (c-index), calibration by calibration plot, and absolute difference in survival (ADS) between the lowest-and the highest-risk groups. RESULTS: The cohort of 292 patients (median age 63 years and median follow-up 3.6 years) had 131 progressions and 96 deaths. The 2-y PFS was 63%. The c-indices were NCCN-IPI 0.6216, R-IPI 0.6004 (P = 0.215), and IPI 0.6104 (P = 0.463). The calibration plots of NCCN-IPI and R-IPI showed nearly perfect agreement (moderate strength), while IPI had miscalibrations. The ADSs were NCCN-IPI 52%, R-IPI 42%, and IPI 25%. CONCLUSION: NCCN-IPI is the best prognostic index compared to IPI and R-IPI in prior studies. However, the prognostic model for DLBCL patients treated with R-CHOP requires updating or integrating biomarkers to improve discrimination to the acceptable level (c-index 0.7).

Spontaneous heparin-induced thrombocytopaenia with adrenal haemorrhage following orthopaedic surgery: a case report and literature review.

A 68-year-old woman was admitted to the hospital for elective total knee arthroplasty in both knees without preceding heparin exposure. She developed adrenal haemorrhage and thrombocytopaenia on postoperative day 12, followed by right leg arterial occlusion and multiple venous intra-abdominal sites thrombosis. After given unfractionated heparin to treat arterial occlusion, platelet count was gradually declined. Spontaneous heparin-induced thrombocytopaenia was diagnosed by heparin-induced platelet activation test with light transmission aggregometry. The patient was successfully treated with fondaparinux and intravenous immunoglobulin. Apixaban was given after recovery of platelet count. Resolution of both thrombus along aorta and adrenal haemorrhage were shown by CT of whole abdomen after 2 months of treatment. Our case demonstrates that this serious complication is important but seldom recognised early.

Types, Clinical Features, and Survival Outcomes of Patients with Acute Myeloid Leukemia in Thailand: A 3-Year Prospective Multicenter Study from the Thai Acute Leukemia Study Group (TALSG).

BACKGROUND: Acute myeloid leukemia (AML) is a common, challenging hematologic malignancy worldwide. Thai data on its characteristics and outcomes have never been systematically reported, to our knowledge. The objective of this study was to determine the clinical features and outcomes of Thai patients with AML. PATIENTS AND METHODS: This was a prospective observational study of nine academic hospitals. Patients with newly diagnosed AML were invited to register online. RESULTS: A total of 679 patients with AML were included. The presence of circulating peripheral blood blasts was correlated with a high white blood cell count. Acute promyelocytic leukemia (APL) had predominantly lower white blood cell counts and higher proportions without peripheral blood blasts compared with non-APL AML. Disseminated intravascular coagulation was commonly presented in APL (37.7%). Splenomegaly and normal platelet count were more frequently seen in patients with Philadelphia chromosome-positive AML. The median follow-up time for those who survived more than 1 year was 28.0 months. One-year overall survival rates for non-APL AML and APL were 31.9% and 88.2%, respectively; 2-year overall survival rates were 29.6% and 88.2%, respectively. Hematopoietic stem cell transplantation could improve survival in non-APL AML. CONCLUSION: APL should be considered despite absence of peripheral blood blast. This study demonstrates poor outcome of Thai AML and more research to improve outcomes are underway. Expanding access to hematopoietic stem cell transplantation should be considered in Thailand.

Event-free survival at 12 months is a strong surrogate endpoint for stage 1 diffuse large B cell lymphoma: a report from Nation Wide Registry Thai Lymphoma Study Group.

Event-free survival at 12 months (EFS12) is a surrogate endpoint for long-term outcomes in many histologic lymphoma subtypes. However, most reports have primarily investigated the implication of EFS12 in advanced-stage non-Hodgkin lymphoma (NHL). There are limited data regarding the significance of EFS12 in early-stage NHL. Herein, we evaluated the prognostic significance of EFS12 in patients with stage 1 diffuse large B-cell lymphoma (DLBCL). Out of 282 patients with stage 1 DLBCL who received intensive therapy, 227 (80.5%) achieved EFS12. The 4-year overall survival (OS) was 91.4% and 4.0% for patients who achieved and failed to achieve EFS12, respectively. Multivariable analyses demonstrated response to treatment and achievement of EFS12 as independent predictors for OS. In conclusion, our study demonstrated EFS12 as a powerful prognostic factor for stage 1 DLBCL. Further validation in more extensive prospective studies is warranted.

Characteristics, treatment patterns, prognostic determinants and outcome of peripheral T cell lymphoma and natural killer/T cell non-Hodgkin Lymphoma in older patients: The result of the nationwide multi-institutional registry Thai Lymphoma Study Group.

INTRODUCTION: Peripheral T cell NHL (PTCL) and natural killer/T cell NHL (NKTCL) are relatively rare disorders. Data on clinical presentation, treatment and outcome are limited especially in older age groups. METHODS: We identified 127 patients with PTCL and NKTCL, excluding cutaneous T/NK cell lymphoma, aged over 60 years old from Thailand nationwide multicenter registry. RESULTS: Of 127 patients, median age of diagnosis was 67 years old. Patients aged older than 75 years old had similar characteristics to younger (60-74 years old) but higher comorbidity index. Seventy-nine patients (62.2%) received intensive/definite multi-agent chemotherapy, however, the proportion was significant lower in older patients (70.4% vs 34.5%, p < .001). After a median follow up duration of 17.3 months, 2-year progression free survival and overall survival were 38.1% and 48.5%. Univariate and multivariable analysis demonstrated older age, poor performance status and absence of definite multi-agent chemotherapy were associated with inferior survival. Definite multi-agent lymphoma specific chemotherapy was an independent factor for overall survival after adjustment for age, comorbidity index, performance status and prognostic index for T cell lymphoma. CONCLUSION: Despite overall poor prognosis of PTCL and NKTCL in older adults, chemotherapy could result in objective response and long-term survival in selected patients of this vulnerable age group thus emphasizing the importance of comprehensive geriatric evaluation.

Event free survival at 24 months is a strong surrogate prognostic endpoint of peripheral T cell lymphoma.

Event free survival at 24 months (EFS24) has been described as a powerful predictor for outcome in several subtypes of B cell lymphoma. However, it was limitedly described in T cell lymphoma. We explored the implication of EFS24 as a predictor marker for peripheral T cell lymphoma (PTCL). We reviewed 293 systemic PTCL patients at 13 nationwide major university hospitals in Thailand from 2007 to 2014. The median event free survival (EFS) and overall survival (OS) of PTCL patients in our cohort was 16.3 and 27.7 months with corresponding 2-year EFS and 2-year OS of 45.8% and 51.9%, respectively. A total of 118 patients achieved EFS24 (no events during the first 24 mo). Patients who achieved EFS24 had better OS than patients who did not (2-y OS 92% vs 18.8%; HR, 0.1; P < .001). The standardized mortality ratio of patients achieving EFS24 was 18.7 (95% CI, 14.6-22.8). Multivariable analysis demonstrated performance status, histologic subtype, remission status, and EFS24 achievement as independent predictors for OS. Our study affirmed the value of EFS24 as a powerful prognostic factor for PTCL. Further validation in prospective study setting is warranted.

Improved Survival of Elderly-fit Patients With Acute Myeloid Leukemia Requiring Intensive Therapy: 3-Year Multicenter Analysis From TALWG.

BACKGROUND: Elderly patients with acute myeloid leukemia (AML) have a poorer prognosis than younger ones. Several factors contribute to the poor outcomes for this patient group. PATIENTS AND METHODS: This study investigated the epidemiology, clinical characteristics, treatment, and clinical outcomes of elderly Thai patients with AML. This 3-year, prospective, multicenter study was focused on Thai patients with AML aged over 60 years who were diagnosed between 2014 and 2016. RESULTS: Of 680 patients with AML, 235 elderly patients with AML (34.6%) were identified, with a mean age of 70 ± 8 years. Using a 3-group cytogenetic risk classification (favorable, intermediate, and adverse risk), the proportions of patients in each category were 3.6%, 73.8%, and 22.6%, respectively. The median follow-up time for surviving patients was 846 days. The median overall survival (OS) of the patients was 128.2 days (range, 0-1205 days), with a 1-year OS of 13%. From a multivariate analysis, the significant factors associated with an improved long-term OS were patients with an Eastern Cooperative Oncology Group performance status 0 to 2 and those receiving intensive therapy. CONCLUSION: Our study confirms the high prevalence of AML in elderly patients with generally poor outcomes. Selected patients with a good performance status and those who received intensive induction treatment could have a long-term survival.

Secondary central nervous system relapse in diffuse large B cell lymphoma in a resource limited country: result from the Thailand nationwide multi-institutional registry.

Secondary central nervous system (CNS) relapse is a serious and fatal complication of diffuse large B cell lymphoma (DLBCL). Data on secondary CNS (SCNS) relapse were mostly obtained from western countries with limited data from developing countries. We analyzed the data of 2034 newly diagnosed DLBCL patients enrolled into the multi-center registry under Thai Lymphoma Study Group from setting. The incidence, September 2006 to December 2013 to represent outcome from a resource limited pattern, management, and outcome of SCNS relapse were described. The 2-year cumulative incidence (CI) of SCNS relapse was 2.7 %. A total of 729, 1024, and 281 patients were classified as low-, intermediate-, and high-risk CNS international prognostic index (CNS-IPI) with corresponding 2-year CI of SCNS relapse of 1.5, 3.1, and 4.6 %, respectively (p < 0.001). Univariate analysis demonstrated advance stage disease, poor performance status, elevated lactate dehydrogenase, presence of B symptoms, more than one extranodal organ involvement, high IPI, and high CNS-IPI group as predictive factors for SCNS relapse. Rituximab exposure and intrathecal chemoprophylaxis offered no protective effect against SCNS relapse. At the time of analysis, six patients were alive. Median OS in SCNS relapsed patients was significantly shorter than relapsed patients without CNS involvement (13.2 vs 22.6 months) (p < 0.001). Primary causes of death were progressive disease (n = 35, 63.6 %) and infection (n = 9, 16.7 %). In conclusion, although the incidence of SCNS relapse in our cohort was low, the prognosis was dismal. Prophylaxis for SCNS involvement was underused even in high-risk patients. Novel approaches for SCNS relapse prophylaxis and managements are warranted.

Accuracy of Bone Marrow Flow Cytometry Analysis in Patients With Plasma Cell Neoplasm in Thailand: A Single Institutional Study.

BACKGROUND: Plasma cell neoplasm is a common hematologic malignancy. Treatment with novel agents results in favorable outcomes. Reliable investigations are required to monitor the residual disease, especially after such effective treatments. Flow cytometric analysis is a speedy and accurate method to detect abnormal cells. The aim of this study was to determine diagnostic performance of flow cytometry in the detection of abnormal plasma cells in bone marrow specimens. MATERIALS AND METHODS: We included bone marrow samples taken from patients suspected to harbor plasma cell neoplasm at the time of diagnosis or follow-up after treatment from 2013 to 2015. Flow cytometric analyses, using cluster of differentiation (CD)19/CD20/CD27/CD38/CD45/CD56/CD117/CD138 and cytoplasmic κ/λ, were done and results compared with morphologic evaluation of marrow aspirate smear, histology, and immunohistochemistry of marrow biopsy and protein electrophoretic analyses. RESULTS: A total of 154 specimens were included. Plasma cell neoplasm was detected in 56 samples (36.4%). Most abnormal plasma cells in this study were CD19-negative (CD19(-))/CD20(-)/CD27(+)/CD38(+)/CD45(-) (or weakly positive)/CD56(+)/CD117(+)/CD138(+). Light chain restriction was found only in 18 samples (32.1%). Sensitivity and specificity of flow cytometric analysis were 91.1% and 96.9%, respectively. For the follow-up cohort, sensitivity and specificity were 86.7% and 66.7%, respectively. CONCLUSION: Analysis of plasma cell neoplasm using flow cytometry has high sensitivity and specificity. As an adjunct to marrow histology and immunohistochemistry, flow cytometry can be used in diagnosis of plasma cell neoplasm and more importantly in monitoring the disease after treatment. We propose a limited panel of CD19/CD38/CD45/CD56/CD117/CD138 for detecting minimal residual disease in Thai patients.

The Possibility of Developing Screening Criteria for Patients Suspected Multiple Myeloma (MM) at Thammasat University Hospital.

Background: Patients suspected with multiple myeloma (MM) are often delayed investigation and diagnosis because of multiple factors. The objective of this study was to evaluate factors leading to diagnosis of MM would help in early diagnosis and treatments. Objective: To evaluate the effectiveness of factors used for myeloma screening and to find any factors that can predict the diagnosis of multiple myeloma. Material and Method: From Aug 2013 to Mar 2015, 300 patients were enrolled into this study. Every patient must have met the criteria of: 1) age above 50 years, 2) at least one of laboratory criteria (Hb <10 g/dL, serum creatinine >2 mg/dL, serum calcium >10 mg/dL), and 3) at least one skeletal criterion (bone pain, osteoporosis or history of fracture). Eligible patients' sample were sent for serum protein electrophoresis (SPEP) to screen for MM. Results: From 300 patients, there were 70 patients (23.3%) diagnosed with MM. Of the 3 criteria, all patients (n = 300, 100%) had age above 50 years as inclusion criteria, mean age was 73 years, 273 cases (91%) had Hb below 10 g/dL, 83 cases (27.66%) had serum creatinine Conclusion: Among many factors those are the common presentation of MM, there were no factor or combination of factors that shown statistically significant to predict the diagnosis of multiple myeloma. However, we would suggest thinking of MM in elderly patients who have bone pain or osteoporosis, hypercalcemia, and azothemia or renal failure.

Situational analysis of palliative care education in thai medical schools.

OBJECTIVE: The Thai Medical School Palliative Care Network conducted this study to establish the current state of palliative care education in Thai medical schools. METHODS: A questionnaire survey was given to 2 groups that included final year medical students and instructors in 16 Thai medical schools. The questionnaire covered 4 areas related to palliative care education. RESULTS: An insufficient proportion of students (defined as fewer than 60%) learned nonpain symptoms control (50.0%), goal setting and care planning (39.0%), teamwork (38.7%), and pain management (32.7%). Both medical students and instructors reflected that palliative care education was important as it helps to improve quality of care and professional competence. The percentage of students confident to provide palliative care services under supervision of their senior, those able to provide services on their own, and those not confident to provide palliative care services were 57.3%, 33.3%, and 9.4%, respectively. CONCLUSIONS: The lack of knowledge in palliative care in students may lower their level of confidence to practice palliative care. In order to prepare students to achieve a basic level of competency in palliative care, each medical school has to carefully put palliative care content into the undergraduate curriculum.

Approximate iatrogenic blood loss in medical intensive care patients and the causes of anemia.

BACKGROUND: Anemia is the most common hematologic problem worldwide especially in developing world. There are many causes of anemia in ICU patient such as gastrointestinal bleeding, anemia of chronic inflammation, trauma or surgery. Patient who were admitted in the ICU need to have a lot of blood tests related to their illness and for further treatment, however that can cause decrease of hematocrit or anemia. OBJECTIVE: To identify the estimate volume of blood drawn from medical ICU patients and find out whether the volume of blood drawn effects the Hemoglobin/Hematocrit (Hb/Hct) or anemia and to identify the cause of anemia in ICU patient. MATERIAL AND METHOD: This is an observation prospective cohort study collecting the volume of blood drawn from patients who were admitted to ICU Thammasat university hospital during 1st October 2007 through 31st March 2008. RESULTS: This study found the mean volume of blood samples drawn from ICU patients is 77.8 ml. Sixty-eight percents of patients had a decrease Hb/Hct level during admission and around sixteen percent had anemia during admission. There is no statistically significant between the volume of blood drawn and the decrease of Hb/Hct. CONCLUSION: Decrease of Hb/Hct or anemia are common in ICU patient. This study showed that iatrogenic blood loss for laboratory investigation is one of the cause of anemia. We concluded that physician should order phlebotomy for necessary investigation for the most effective of diagnosis and treatment, but the less complication.

NK/T cell lymphoma, nasal type with sinonasal mass and palatal ulcer: a clinical case report and review of treatment.

Extranodal Nasal NK/T cell lymphoma, relatively uncommon type of non-Hodgkin's lymphoma, is highly aggressive disease with poor outcomes. Early stage disease may response to radiotherapy alone. However the treatments are usually chemotherapy and radiotherapy combination and late stage disease may not response to any available therapy. We reported a 45-year-old woman with extranodal nasal type NK/T cell lymphoma. The patient presented with chronic nasal stuffiness and mucous bloody discharge. The nasal mass is extensively involved periorbital and sinonasal soft tissue. Due to the extensively involved of tumor, combination chemotherapy was used to induce response. This patient was involved by complicated infection and palatal ulcer with likely perforation. The salvage chemotherapy was given and the treatment of NK/T cell lymphoma from other reports and literatures were reviewed.

Natural killer cell malignancy associated with Epstein-Barr virus and hemophagocytic syndrome.

Natural killer cell malignancy is a rare and aggressive lymphoid neoplasm encompassing extra-nodal NK/T-cell lymphoma, nasal-type (ENKLN) and aggressive NK-cell lymphoma/leukemia (ANKL). A case of cutaneous ENKLN and a case of ANKL in Thai patients are reported Both patients developed hemophagocytic syndrome and shortly succumbed to death. The cells in cutaneous ENKLN are small to medium in size with minimal cytoplasm, round nuclei, irregular nuclear membrane, andfine chromatin with inconspicuous nucleoli. While that of ANKL are medium to large-sized mononuclear cells with moderate cytoplasm. Their nuclei are elongated to embryo-like with irregularly thickened nuclear membrane, fine chromatin, and small to occasional prominent nucleolus. Ancillary techniques studied on paraffin embedded tissues of both cases demonstrated that the neoplastic cells exhibit cytoplasmic CD3+, CD56+ and cytotoxic granules + by immunohistochemistry, absence of T cell receptor gene rearrangement by PCR, and presence of Epstein-Barr virus mRNA (EBER) transcripts by in situ hybridization. The authors reviewed the literature on natural killer cell neoplasm and compared the clinical characteristics, natural history, and association of Epstein-Barr virus infection with hemophagocytic syndrome.

IgA nephropathy associated with Hodgkin's disease in children: a case report, literature review and urinary proteome analysis.

We report herein a rare case of IgAN associated with Hodgkin's disease in a 14-year-old boy. Clinical manifestations and laboratory parameters were improved after treatment with CHOP chemotherapy and enalapril. Urinary proteins were isolated and examined using state-of-the-art proteomic technology, before and during the treatment course. Two-dimensional gel electrophoresis showed obvious alterations in the urinary proteome profile in response to such therapy. Quantitative intensity analysis and gel mapping revealed 14 altered proteins with reduced excretion levels during the treatment course, including albumin, albumin complexed with decanoic acid, alpha-1 antitrypsin, cadherin-11 precursor, collagen alpha 1 (VI) chain precursor, complement C1q tumor necrosis factor-related protein, Ig heavy chain, Ig light chain, kininogen, inter-alpha-trypsin inhibitor (alpha-1 microglobulin), inter-alpha-trypsin inhibitor heavy chain, leucine-rich alpha-2 glycoprotein, beta-2 microglobulin, and transferrin precursor. Their potential roles in the pathogenesis and pathophysiology of IgAN are discussed.