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Transfusion-dependent thalassemia (TDT) is often accompanied by complications related to iron overload and the development of malignant solid tumors or hematological malignancies. The occurrence of Neuroendocrine carcinoma, specifically in the respiratory tract, is very rare, with a prevalence of approximately 25%. Therefore, this study presented a case of a 42-year-old male with a beta-thalassemia major at 28 years, complaining of shortness of breath. This case was reported due to its rarity in providing information about solid tumors in thalassemia patients. The physical examination revealed several symptoms, including tachycardia, tachypnea, anemia, icteric sclera, elevated jugular venous pressure, coarse wet Ronchi in the medial to basal areas of both lungs, hepatomegaly, and splenomegaly (Schuffner 4). The patient regularly received blood transfusions and iron chelation therapy. A thoracic CT scan showed a lung mass and a biopsy of the mass revealed Pulmonary Neuroendocrine Carcinoma with high-grade proliferation and, large cell type. The patient also passed through cisplatin-etoposide chemotherapy for 6 cycles every 21 days. There is almost no data on pulmonary neuroendocrine carcinoma in thalassemia patients, so it is hoped that this case report can provide information about malignant solid tumors that can occur in thalassemia patients.

Background: Osteoporosis is a major problem in transfusion-dependent thalassemia patients (TDT) patients. Osteoprotegerin (OPG) is one of several bone markers that are closely associated with osteoporosis in TDT patients. OPG is a glycoprotein that functions as a feedback receptor for the Receptor Activator of Nuclear Factor kappa B Ligand (RANKL), which is an alpha tumor necrosis factor receptor. One of the causes of decreased bone mass density is iron toxicity, which can be identified by showing elevated transferrin saturation. Bone mass dual X-ray absorptiometry (DEXA) is a gold standard for the diagnosis of osteoporosis, these procedures are not commonly available in Indonesia. This study was conducted to analyze the correlation between serum levels of OPG and transferrin saturation in TDT patients., Methods: A correlational study with a cross-sectional approach analyzed data from TDT patients at Hemato-Oncology Medic Outpatient Clinic, Hasan Sadikin General Hospital, Bandung, Indonesia. Primary data were obtained through blood sampling and anthropometry measurement while secondary data were obtained from the patient's medical records. OPG and transferrin saturation levels were assessed using the ELISA method. Research data were analyzed using the rank Spearman correlation test., Results: Data were collected from 51 research subjects (30 women dan 21 men). The median OPG level was 380 (170-1230) pg/mL and the median transferrin saturation level was 89.4 (66.7 - 96.2)%. Analysis of correlation showed a significant correlation between and transferrin saturation level with a coefficient value of r -0.539 and p-value <0.001., Conclusion: There was a significant inverse correlation between OPG with transferrin saturation in TDT patients.

POEMS syndrome is defined by the presence of a peripheral neuropathy (P), a monoclonal plasma cell disorder (M), and other paraneoplastic features, the most common of which include organomegaly (O), endocrinopathy (E), skin changes (S). Not all features of the disease are required to make the diagnosis. We report a case of POEMS syndrome in a 50-year-old female who presented with weakness, abdominal swelling and history of red cell transfusions. Because of the hepatosplenomegaly (Schuffner VII) we diagnosed her as chronic myelocytic leukemia (CML) or myelofibrosis. Her peripheral blood smear did not show granulopoiesis maturation from myeloblast nor leukoerytroblastic feature which was characteristic of CML and myelofibrosis. We found also anemia, peripheral motoric neuropathy and hyperpigmentation at her legs. The protein electrophoresis showed monoclonal gammopathy on b2 globulin. Bone marrow examination showed normal plasma cells. There was no lytic or sclerotic lesion on Schedel and tibia x-ray. The echocardiography showed pulmonary hypertension, pulmonary regurgitation, right and left ventricle hypertrophy with normal ejection fraction (50%). She was treated with melphalan 10 mg/m2 (day 1-4) and prednisone 60 mg/m2 (day 1-4) every 6 weeks with packed red cells transfusion. After 3 cycles the monoclonal protein was reduced from 35.5% to 26.1% (normal 2-5%) without changes in her spleen size. Until then she continued on melphalan and prednisone treatment. Although POEMS syndrome is a rare disease, it should be considered in patient with hepatosplenomegaly, especially if accompanied by peripheral neuropathy.