Hepatosplenic Hodgkin lymphoma without lymphadenopathy following reversible methotrexate-associated lymphoproliferative disorder.

Lymphoproliferative disorders (LPDs) occur more frequently in rheumatoid arthritis (RA) patients treated with immunosuppressive agents than in the non-RA population. However, the various forms of disease progression have not yet been elucidated in detail. We encountered a case of Epstein-Barr virus (EBV)-positive atypical polymorphous LPD in the cervical and intraabdominal lymph nodes with hepatosplenomegaly in an 88-year-old female with RA who had taken infliximab and methotrexate (MTX) for six years. Although spontaneous remission occurred following the withdrawal of infliximab and MTX, reversible LPD evolved into hepatosplenic Hodgkin lymphoma without lymphadenopathy presenting as a cholestatic febrile illness. Our findings suggest that the recurrent lesions of MTX-associated LPDs may not always coincide with the primary lesion and may present unexplained findings based on various extranodal diseases.

Disseminated tuberculosis after pregnancy progressed to paradoxical response to the treatment: report of two cases.

BACKGROUND: Early postpartum women are more likely to develop tuberculosis than nonpregnant women mainly due to immune reconstitution after delivery. Paradoxical response (PR) during antituberculosis treatment also arises via recovery from immunosuppression. However, no study focused on PR during antituberculosis treatment in a postpartum patient has been reported. CASE PRESENTATION: We present two sequential cases (Patient 1: 26-year-old; Patient 2: 29-year-old) of postpartum tuberculosis with pulmonary and extrapulmonary lesions (Patient 1: peritonitis; Patient 2: psoas abscess secondary to spondylitis). Both cases progressed to PR (worsening of pre-existing lung infiltrations (Patients 1, 2) and new contralateral effusion (Patient 2)) in a relatively short time after initiation of treatment (Patient 1: 1 week; Patient 2: 3 weeks), suggesting that immune modulations during pregnancy and delivery may contribute to the pathogenesis of both disseminated tuberculosis and its PR. The pulmonary lesions and effusion of both cases gradually improved without change of chemotherapy regimen. CONCLUSION: Physicians should recognize PR in tuberculosis patients with postpartum and then evaluate treatment efficacy.

[Exacerbation of cranial nerurological symptoms by platelet transfusion before the diagnosis of thrombotic thrombocytopenic purpura].

A 47-year-old woman was transported to our hospital because of vomiting and syncope after breakfast. Physical examination revealed icterus and anemia of bulbar conjunctivas, and abnormal neurological findings were detected. Laboratory data indicated marked anemia and thrombocytopenia (Hb 5.2 g/dl, Plt. 0.6×10(4)/µl), but no leukocyte abnormalities were found. Transaminase was slightly elevated, and serum indirect bilirubin in was also elevated. Based on these data, we initially suspected Evan's syndrome, which involves idiopathic thrombocytopenic purpura with autoimmune hemolytic anemia. So we transfused red blood cells, performed platelet transfusion, and administered steroids, but there was no response to these therapies. On the 4th day of admission, she developed a stroke followed by coma. After the stroke, we diagnosed the case as thrombotic thrombocytopenic purpura (TTP) because laboratory findings showed diminished activity of ADAMTS 13 (a disintegrin-like metalloproteinase with thrombospondin type 1 motifs 13) and ADAMTS 13 antigen. It is important to suspect TTP when hemolytic anemia with thrombocytopenia is observed, and to check the activity and antigen of ADAMTS13 immediately for the diagnosis. Platelet transfusion should be done cautiously in these cases.

[A case of pulmonary nontuberculous mycobacteriosis induced by tocilizumab treatment for rheumatoid arthritis].

A 65-year-old woman whose rheumatoid arthritis was treated with tocilizumab (TCZ) was found in chest radiography to have a new consolidation in the right lower lung field. Positive Mycobacterium intracellulare and Mycobacterium avium cultures in sputum and bronchial secretions yielded a diagnosis of pulmonary nontuberculous mycobacteriosis. The most common adverse TCZ effect is infection. This case highlights the fact that those treated with TCZ should be considered at elevated risk for developing nontuberculous mycobacteriosis.

[A case of adult onset idiopathic pulmonary hemosiderosis markedly improved by steroid therapy].

A case of adult onset idiopathic pulmonary haemosiderosis (IPH) was reported. A 53-year-old man was admitted to our hospital because of repeated bloody sputum on June 2, 2006. Chest radiograph on admission disclosed diffuse infiltrative shadows in both lung fields, and one month later these shadows became more marked. The chest CT on July 5, 2006 showed patchy areas of ground-glass opacity and consolidation, exhibiting a distinctly peripheral distribution. Bronchoscopic findings revealed oozing bleeding from the orifice of B5 in the right lung and B9 in the left lung. We employed video-assisted thoracoscopic surgery for lung biopsy and he as primary IPH was diagnosed clinicopathologically. His symptoms and radiographic findings were markedly improved after steroid therapy, followed by no signs of recurrence. It may be important to establish a definitive diagnosis early, even in IPH, using VATS, for further effective therapy.

Non-Hodgkin's lymphoma involving a femur bone and bilateral adrenal glands alone with adrenal insufficiency.

Primary bone lymphoma and primary adrenal lymphoma are rare clinicopathological entities of non-Hodgkin's lymphoma (NHL). We present the first case of diffuse large B-cell lymphoma with the involvement of a single bone and both adrenal glands alone with adrenal insufficiency. As primary extranodal NHL may have other unusual extranodal lesions, which may present unexplained clinical findings, patients with primary extranodal NHL require careful systemic examination, even when lymphadenopathy is absent.

[Transient worsening of chest radiograph and development of lymphadenopathy during chemotherapy for miliary tuberculosis].

A-37-year old woman was referred to our hospital because of bilateral pulmonary micronodular shadows on chest X-ray. Prednisolone was reported to be administered for her coughs and dyspnea more than a month, but was discontinued recently. Under the diagnosis as miliary tuberculosis, we started to treat her with the combined use of pyrazinamide, isoniazid, rifampicin, and ethambutol. Then her symptoms subsided gradually. Two months later, however, high fever developed, followed by exacerbation of the radiographic shadows, and marked cervical and mediastinal lymphadenopathy. We considered them so-called paradoxical worsening, and continued the antituberculosis therapy unchanged. Those clinical manifestations began to subside about 4 months after the initiation of the treatment. Paradoxical worsening has been described as a relatively rare manifestation, and seem to be attributable to prompt recovery of the immunity to mycobacterial antigens after the use of antituberculous therapy. We considered that, in this case, disseminated tuberculosis and firstly administered steroid that might suppress immune function, and discontinuation of steroid therapy followed by the bactericidal antituberculous chemotherapy were associated with the development of the paradoxical reactions, by analogy with immune reconstitution syndrome frequently reported in HIV-related tuberculosis patients.

Pyothorax-associated lymphoma (PAL) with biclonal Epstein-Barr virus infection: characterization of a novel PAL cell line with unique features.

Pyothorax-associated lymphoma (PAL) is a representative form of diffuse large B-cell lymphoma associated with chronic inflammation, in which the Epstein-Barr virus (EBV) genome is consistently detectable in the lymphoma cells of all PAL cases. Cell lines and animal models would be useful for understanding better this rare lymphoma, but reports of PAL-derived cell lines are scarce. We report a new PAL cell line, designated Pal-2, with unique phenotypic expression. Pal-2 is the first PAL cell line that carries a biclonal EBV infection with abundant viral genome and that exhibits tumorigenic capacity once injected into nude mice.

Video-assisted thoracic surgery lobectomy for lung cancer in a patient with complete situs inversus.

Complete situs inversus is a rare abnormality of autosomal recessive inheritance; it requires particular care during surgery, because the viscus anatomy is a mirror image of the normal anatomy. Reports of surgery for lung cancer in cases of complete situs inversus are very rare. Here, we report a case of lobectomy for lung cancer of the right lower lobe performed using video-assisted thoracic surgery (VATS) in a patient with complete situs inversus. We emphasize the importance of careful examination of the relationship between the bronchus, pulmonary artery, and pulmonary vein in the hilum of the lung in cases of complete situs inversus requiring lung resection for cancer; this is even more necessary when VATS is performed.

CD5-positive diffuse large B cell lymphoma infiltrating the central nervous system presenting Guillain-Barré-like syndrome after chemotherapy.

An 83-year-old woman was admitted to our hospital with abdominal pain. Examination revealed mediastinal lymphoadenopathy, hepatosplenomegaly, and infiltration of abnormal cells into the bone marrow with hemophagocytosis, and CD5-positive diffuse large B cell lymphoma was diagnosed. Chemotherapy was administered and progressive weakness of the limbs, resembling a Guillain-Barré-like syndrome, subsequently appeared. Cerebrospinal fluid examination indicated lymphoma cell infiltration. Although immune globulin and steroid therapies were not effective, intrathecal injection of methotrexate, predonisolone, and cytarabine improved these symptoms. Subsequent to chemotherapy, cell surface antigen changes were observed in the cerebrospinal fluid relative to those in bone marrow.

Cancer of the left lung with persistent left superior vena cava.

As operations for lung cancer become more common, more anomalies of various pulmonary arteries and veins are being encountered. Persistent left superior vena cava (PLSVC) is caused by abnormal development of the sinus venosus in early fetal life. In cases of PLSVC with left lung cancer, particular care must be exercised during the excision of the pulmonary vein, focusing on the region into which the PLSVC flows. In such anomalous cases, proper excision of the pulmonary artery and vein and lymph node dissection require a firm grasp of anatomic detail using preoperative computed tomographic scanning.

Acute myeloid leukemia with myelodysplasia-related changes mimicking acute promyelocytic leukemia.

We describe a patient with acute myeloid leukemia with myelodysplasia-related changes (AML-MRC) that clinically resembled acute promyelocytic leukemia (APL). The karyotype of his leukemic cells was 46, XY, del (3) (q?) and did not include a chromosomal translocation involving the retinoic acid receptor-α gene. However, retinoic acid syndrome developed, and partial remission was achieved after treatment with all-trans retinoic acid (ATRA) followed by chemotherapy. Our case might provide new insights into the mechanism of the growth inhibitory effect of ATRA on APL-like cells.

Immunoglobulin D-lambda type multiple myeloma presenting with FDG-PET/CT negative bone marrow involvement.

We here report a rare case of a patient with IgD-lambda-positive multiple myeloma presenting with FDG-PET/CT negative bone marrow involvement. A 72-year-old man was admitted to our hospital for evaluation of a paravertebral tumor of the chest. Thoracotomy was performed and a histopathological evaluation of resected intrathoracic tumor demonstrated a plasmacytic neoplasma. Initially we thought that this case was a solitary plasmacytoma because there were no positive findings on postoperative FDG-PET/CT. However, bone marrow aspiration study demonstrated massive infiltration of myeloma cells (72%). It is necessary to recognize that IgD-lambda type myeloma cells may not be sufficiently metabolically active to form high uptake on FDG-PET/CT.