Primary bone lymphoma (PBL) is an uncommon type of extranodal lymphoma involvement. An anaplastic large-cell lymphoma (ALCL) is an extremely rare type of PBL, and it remains unclear whether ALCLs that primarily involve the bone exhibit favourable or unfavourable biological behaviour, and whether they are similar to ALCLs in general, or not. We reported a case of ALKpositive ALCL with primary bone involvement, and reviewed the clinicopathological features of 22 previously reported cases. An ALCL with primary bone involvement mostly affects younger patients with a preponderant towards the involvement of axial-bone. The prognosis of an ALCL that primarily involves bone is unfavourable, compared with PBL generally. The ALK-positive ALCLs in PBLs had less decedents than the ALK-negative ALCLs with a statistical non-significance (p=0.198).
primary-bone lymphoma

Myelofibrosis results from stimulation of bone marrow stromal fibroblasts by fibrogenic cytokines elaborated by neoplastic or reactive cells in the marrow. Chronic idiopathic myelofibrosis should be differentiated from secondary myelofibrosis resulting from bone marrow involvement of malignant lymphoma because these diseases have different therapeutic strategies. Myelofibrosis in systemic lupus erythematosus is an uncommon but well-recognized complication, and identifying an autoimmune myelofibrosis is important in diagnosing this benign cause of myelofibrosis. We report two cases of myelofibrosis presenting the clinical and radiologic findings that mimicked malignant lymphoma -a case of autoimmune myelofibrosis associated with systemic lupus erythematosus showing extensive lymphadenopathy and a case of chronic idiopathic myelofibrosis with focal intrasplenic extramedullary hematopoiesis- and discuss the importance of the clinical information and radiologic findings for the pathologic diagnosis of myelofibrosis.
Abdomen* ; Bone Marrow ; Cytokines ; Diagnosis ; Fibroblasts ; Hematopoiesis, Extramedullary* ; Lupus Erythematosus, Systemic* ; Lymphatic Diseases* ; Lymphoma* ; Primary Myelofibrosis*

Acute lymphoblastic leukemia (ALL), in general, can be diagnosed by detecting blasts in peripheral blood or bone marrow. Some of the cases of ALL do not show typical leukemic features, and only manifest as refractory anemia, thrombocytopenia, myelofibrosis and lymphocytic infiltration into bone marrow. Several months after presentation, they may reveal typical leukemic features and are diagnosed as ALL. This kind of leukemia is called ALL with aleukemic prodrome. Although the incidence of ALL with aleukemic prodrome is 1.5~2.2% of childhood ALL cases, it is rarely reported in Korea. We experienced a 6 month-old female infant who presented with refactory anemia and thrombocytopenia, and two serial of bone marrow examination revealed only myelofibrosis. She subsequently developed ALL 3 months later. We report this case with a brief review of related literatures.
Anemia ; Anemia, Refractory ; Bone Marrow ; Bone Marrow Examination ; Female ; Humans ; Incidence ; Infant ; Korea ; Leukemia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Primary Myelofibrosis ; Thrombocytopenia

Brown tumors also called as osteoclastomas, are rare nonneoplastic lesions that arise in the setting of primary or secondary hyperparathyroidism. Parathyroid adenomas or hyperplasia constitute the major Brown tumor source in primary hyperparathyroidism while chronic renal failure is the leading cause in secondary hyperparathyroidism. Most of the patients with the diagnosis of primary hyperparathyroidism present with kidney stones or isolated hypercalcemia. However, nearly one third of patients are asymptomatic and hypercalcemia is found incidentally. Skeletal involvement such as generalized osteopenia, bone resorption, bone cysts and Brown tumors are seen on the late phase of hyperparathyroidism. The symptoms include axial pain, radiculopathy, myelopathy and myeloradiculopathy according to their locations. Plasmocytoma, lymphoma, giant cell tumors and metastates should be ruled out in the differential diagnosis of Brown tumors. Treatment of Brown tumors involve both the management of hyperparathyroidism and neural decompression. The authors report a very rare spinal Brown tumor case, arisen as the initial manifestation of primary hyperparathyroidism that leads to acute paraparesis.
Bone Cysts ; Bone Diseases, Metabolic ; Bone Resorption ; Decompression ; Diagnosis ; Diagnosis, Differential ; Giant Cell Tumors ; Humans ; Hypercalcemia ; Hyperparathyroidism ; Hyperparathyroidism, Primary* ; Hyperparathyroidism, Secondary ; Hyperplasia ; Kidney Calculi ; Kidney Failure, Chronic ; Lymphoma ; Paraparesis ; Parathyroid Neoplasms ; Plasmacytoma ; Radiculopathy ; Spinal Cord Diseases ; Spine*

Myelofibrosis is a myeloproliferative neoplasm characterized by abnormal bone marrow megakaryocyte proliferation with reticulin and collagen fibrosis, leukoerythroblastosis, anemia, increased level of serum lactate dehydrogenase and splenomegaly. Myelofibrosis associated with malignant lymphoma is rare and survival rates appear to have been poor. Herein, we describe our experience in a patient who remained in complete remission with high-dose therapy (HDT) with autologous peripheral blood stem cell transplantation (PBSCT) for ALK-negative ALCL presenting with rapidly progressing myelofibrosis.
Anemia ; Bone Marrow ; Collagen ; Fibrosis ; Humans ; L-Lactate Dehydrogenase ; Lymphoma ; Lymphoma, Large-Cell, Anaplastic ; Megakaryocytes ; Peripheral Blood Stem Cell Transplantation ; Primary Myelofibrosis ; Reticulin ; Splenomegaly ; Stem Cell Transplantation ; Survival Rate

During the last decade the issue of therapy-related myelodysplasia (t-MDS) and acute myeloid leukemia (t-AML) following high-dose chemotherapy (HD-CT) and autologous stem cell transplantation (ASCT) or conventional chemotherapy, radiotherapy for malignant diseases has become increasingly important. The number of patients with hematologic malignancies and chemosensitive or radiosensitive solid tumors undergoing this new type of treatment has expanded dramatically. Recently, we experienced a case of 35-year-old female patient with therapy-related acute lymphoblastic leukemia (t-ALL) after 131I treatment for thyroid papillary carcinoma. Total 131I dose the patient received was 900mCi and patient was diagnosed as having ALL 11 months after last 131I treatment. Although the prognosis of therapy-related acute leukemia/myelodysplastic syndrome is poor, the patient is alive in complete remission after allogeneic bone marrow transplantation from HLA-matched sibling donor. Given the rarity of this case, we report the case with literature reviews.
Adult ; Bone Marrow Transplantation ; Carcinoma, Papillary* ; Drug Therapy ; Female ; Hematologic Neoplasms ; Humans ; Leukemia, Myeloid, Acute ; Neoplasms, Second Primary ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Prognosis ; Radiotherapy ; Siblings ; Stem Cell Transplantation ; Thyroid Gland* ; Thyroid Neoplasms ; Tissue Donors

We experienced a case of non-Hodgkin's lymphoma presented only as right side pleural effusion, that is primary effusion lymphoma (PEL), in a 75 year-old male patient in Korea where is the endemic area of tuberculosis. He visited our hospital complaining of exertional dyspnea. He did not have B symptoms. The breathing sound was decreased on the right side chest, but we could not find external lymphadenopathy or hepatosplenomegaly on physical examination. Simple chest radiograph showed right side pleural effusion. The cells of pleural fluid were lymphocyte-predominant and the pH, protein, lactate dehydrogenase, adenosine deaminase of the fluid was 7.31, 38 g/L, 381 U/L, 31 U/L, respectively. The biopsy specimen of the parietal pleura was diagnosed as non-Hodgkin's lymphoma of small lymphocytic type. Computed tomograph of the chest, abdomen and pelvis, and the biopsy of bone marrow were negative for disease. We tried up to 3 cycles of chemotherapy with adriamycin, vincristine, cyclophosphamide and prednisolone and there was a marked decrease in the amount of the pleural effusion.
Abdomen ; Adenosine Deaminase ; Aged ; Biopsy ; Bone Marrow ; Cyclophosphamide ; Doxorubicin ; Drug Therapy ; Dyspnea ; Humans ; Hydrogen-Ion Concentration ; Korea ; L-Lactate Dehydrogenase ; Lymphatic Diseases ; Lymphoma ; Lymphoma, Non-Hodgkin* ; Lymphoma, Primary Effusion ; Male ; Pelvis ; Physical Examination ; Pleura ; Pleural Effusion* ; Prednisolone ; Radiography, Thoracic ; Respiratory Sounds ; Thorax ; Tuberculosis ; Vincristine

Hepatocellular cell carcinoma (HCC) is one of the most common and malignant neoplasm in the world. Waldenstrom's macroglobulinemia (WM) is a rare B-lymphocyte neoplasia characterized by monoclonal production of IgM Igs and by a marrow containing a diffuse infiltrate of plasmocytoid lymphocytes. A 67-year-old man was admitted because of multiple site pain, especially right flank and posterior neck pain. Physical examination showed hepatomegaly. Laboratory findings were as follows; total protein 8.6 g/dL, albumin 3.1 g/dL with monoclomal protein(IgM-lamda). Hepatitis C virus(HCV) infection was detected by polymerase chain reaction(PCR). Computed tomography and celiac angiography disclosed HCC. Aspiration biopsy of the liver revealed HCC. Magnetic resonance imaging showed compression fracture and epidural mass in cervical spines. Bone marrow examination revealed normocellularity with an increase of lymphoplasmacytic series. He was diagnosed as having WM and HCC. HCC is frequently associated with other malignancies. As the site of the extrahepatic primary cancer, the stomach ranked first. WM is also known for its association with an increased incidence of a second neoplasm, most of which are less differentiated lymphomas. but an association with a non-lymphoreticular malignancy is quite rare. We report first case of synchronous HCC with bone metastasis and WM (IgM,lamdatype) in korean with chronic hepatitis C.
Aged ; Angiography ; B-Lymphocytes ; Biopsy, Needle ; Bone Marrow ; Bone Marrow Examination ; Carcinoma, Hepatocellular* ; Fractures, Compression ; Hepacivirus ; Hepatitis C ; Hepatitis C, Chronic* ; Hepatitis, Chronic* ; Hepatomegaly ; Humans ; Immunoglobulin M ; Incidence ; Liver ; Lymphocytes ; Lymphoma ; Magnetic Resonance Imaging ; Neck Pain ; Neoplasm Metastasis ; Neoplasms, Second Primary ; Physical Examination ; Spine ; Stomach ; Waldenstrom Macroglobulinemia*

: One of the most feared complications of childhood cancer treatment is second malignant neoplasms (SMNs). This study evaluates the incidence, risk factors and outcomes of SMNs in a tertiary paediatric oncology centre in Singapore.: A retrospective review was conducted on patients diagnosed with childhood cancer under age 21 and treated at the National University Hospital, Singapore, from January 1990 to 15 April 2012. Case records of patients with SMNs were reviewed.: We identified 1124 cases of childhood cancers with a median follow-up of 3.49 (0 to 24.06) years. The most common primary malignancies were leukaemia (47.1%), central nervous system tumours (11.7%) and lymphoma (9.8%). Fifteen cases developed SMNs, most commonly acute myeloid leukaemia/myelodysplastic syndrome (n = 7). Median interval between the first and second malignancy was 3.41 (0.24 to 18.30) years. Overall 20-year cumulative incidence of SMNs was 5.3% (95% CI, 0.2% to 10.4%). The 15-year cumulative incidence of SMNs following acute lymphoblastic leukaemia was 4.4% (95% CI, 0% to 8.9%), significantly lower than the risk after osteosarcoma of 14.2% (95% CI, 0.7% to 27.7%) within 5 years (<0.0005). Overall 5-year survival for SMNs was lower than that of primary malignancies.: This study identified factors explaining the epidemiology of SMNs described, and found topoisomerase II inhibitor use to be a likely risk factor in our cohort. Modifications have already been made to our existing therapeutic protocols in osteosarcoma treatment. We also recognised the importance of other risk management strategies, including regular long-term surveillance and early intervention for detected SMNs, to improve outcomes of high risk patients.
Bone Neoplasms ; therapy ; Cancer Care Facilities ; Central Nervous System Neoplasms ; therapy ; Follow-Up Studies ; Humans ; Incidence ; Leukemia ; therapy ; Leukemia, Myeloid, Acute ; epidemiology ; Lymphoma ; therapy ; Myelodysplastic Syndromes ; epidemiology ; Neoplasms ; therapy ; Neoplasms, Second Primary ; epidemiology ; Osteosarcoma ; therapy ; Pediatrics ; Retrospective Studies ; Risk Factors ; Singapore ; epidemiology ; Survivors ; statistics & numerical data ; Tertiary Care Centers ; Time Factors ; Topoisomerase II Inhibitors ; therapeutic use

Treatment-related myelodysplastic syndrome (t-MDS) and acute myelogenous leukemia (t-AML) are now well established as complications of cytotoxic chemotherapy. We experienced a 28-yr-old female patient who developed t-MDS/t-AML with characteristic chromosomal abnormalities including 11q23 chromosomal rearrangement following high-dose chemotherapy with autologous stem cell transplantation (ASCT) for non-Hodgkin's lymphoma. The patient was admitted with bulky abdominal masses of B cell lineage non-Hodgkin's lymphoma. After 2 cycles of systemic chemotherapy of the Vanderbilt regimen, the patient underwent ASCT with high dose chemotherapy of the BEAC regimen. She also received radiation of 48 Gy for the residual periportal lymphadenopathy. The initial cytogenetic analysis of the infused mononuclear cells revealed a normal karyotype. Twenty two months after the ASCT, pancytopenia was noted and her bone marrow aspirate showed dysplastic hemopoiesis with myeloblasts up to 12% of nonerythroid nucleated cells. The patient was diagnosed as t-MDS (refractory anemia with an excess of blasts). Cytogenetic analysis showed complex chromosomal abnormalities including 11q23 rearrangement, which is frequently found in topoisomerase II inhibitor-related hematologic malignancies. Four months later, it was noted that the t-MDS had evolved into an overt t-AML. Cytogenetic analysis showed an evolving pattern with more complex abnormalities. The patient was treated with combination che-motherapy, but her leukemic cells were resistant to the therapy.
Adult ; Antineoplastic Agents, Phytogenic/adverse effects ; Antineoplastic Combined Chemotherapy Protocols/*adverse effects ; B-Lymphocytes/cytology ; Bone Marrow Cells/pathology ; Carmustine/*adverse effects ; Chromosome Aberrations ; Chromosomes, Human, Pair 11 ; Combined Modality Therapy/adverse effects ; Cyclophosphamide/*adverse effects ; Cytarabine/*adverse effects ; Etoposide/*adverse effects ; Female ; Gene Rearrangement ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Humans ; Leukemia, Myeloid, Acute/*etiology/genetics ; Lymphoma, Non-Hodgkin/*therapy ; Myelodysplastic Syndromes/*etiology/genetics ; Neoplasms, Second Primary/*etiology ; Pelvis ; Pregnancy ; Pregnancy Complications, Neoplastic/*therapy ; Transplantation, Autologous