Simultaneous occurrence of a hepatocellular carcinoma and a hepatic non-Hodgkin's lymphoma infiltration

To investigate the simultaneous occurrence of hepatocellular carcinoma and non-Hodgkin's lymphoma, we report the case of a 70 year old patient with a primary diagnosis of non-Hodgkin's lymphoma in 2002. In a routine follow up investigation of his chronic lymphocytic leukemia a newly detected mass in the Couinaud's segments 2 and 3 was found. No hepatitis C virus or hepatitis B virus infection or cirrhosis was evident. After laparoscopic segmentectomy the histological examination revealed a hepatocellular carcinoma. While the relation between liver parenchyma damages and hepatocellular carcinoma or non-Hodgkin's lymphoma is well known, only a few publications have focused on the coexistence of hepatocellular carcinoma and non-Hodgkin's lymphoma. With this case we demonstrate the coexistence of these diseases without having a predamaged liver parenchyma.     

Primary gastric lymphoma associated with human T-cell leukaemia virus I

We herein report a case of primary gastric lymphoma with reference to human T-cell leukaemia virus type I. A 65-year-old male was admitted to our hospital for emergency treatment of abdominal pain and haematemesis. Upper gastrointestinal endoscopy on admission revealed a disintegrated tumour with bleeding in the cardia. Total gastrectomy with distal pancreatomy and splenectomy was performed. The patient soon fell into cachexic condition due to early recurrence of abdominal lymph nodes and a rapid increase of the tumour, and died 80 days after operation. The pathological diagnosis was non-Hodgkin's lymphoma with diffuse large cell type and T-cell lineage. The patient was serologically positive for human T-cell leukaemia virus type I (HTLV-I) antibody. Southern blot analysis on the resected tumour tissue showed a monoclonal integration of HTLV-I pro-viral DNA. These results confirmed the current case to be primary adult T-cell leukaemia/lymphoma of the stomach.     

Acute hepatic failure due to hepatosplenic B-cell non-Hodgkin's lymphoma in a patient infected with hepatitis C virus

We report a 75-year-old Japanese man infected with hepatitis C virus (HCV) who died of acute hepatic failure due to the hepatic infiltration of B-cell non-Hodgkin's lymphoma (NHL) cells. He suddenly developed jaundice, fatigue, fever, and hepatosplenomegaly during the course of chronic infection with HCV. Postmortem liver necropsy revealed extensive infiltration of lymphoma cells into the liver. Although the association between HCV infection and NHL has recently become a matter of concern, we believe this to be the first reported case of acute hepatic failure caused by hepatic involvement of non-Hodgkin's lymphoma in an HCV-infected patient. (Received Jan. 12, 1998; accepted June 26, 1998)     

Non-Hodgkin's lymphoma in an elderly patient complicated by cyclophosphamide-induced allergic hepatic dysfunction]

A 77-year-old male presented at our Department of Urology in August 1990 with a gradually enlarging swelling in the right scrotum. On August 21, right high orchietomy was performed. This was diagnosed histologically as non-Hodgkin's lymphoma (diffuse large cell type), and the patient was transferred to our department on September 11 for further examination and treatment. As enlargement of the lymph nodes around the abdominal aorta was evident, it was diagnosed as stage IIA according to the Ann Arbor Classification. Beginning on September 21, three courses of COP-BLAM therapy (CPM, VCR, PDN, BLM, ADR, PCZ) were administered (the third course started on November 8) to achieve complete remission. Hepatic dysfunction appeared, however, from November 16, and by November 19, GOT and GPT increased to 6700 and 2120, respectively, with aggravation of jaundice. PDN therapy was instituted, and GOT and GPT improved gradually, but jaundice did not improve. On December 22, laparoscopy was performed, and liver biopsy produced histologic findings of drug-induced hepatitis. Further, lymphoblastic response was positive for CPM. Hepatic failure occurred on December 29, and plasma exchange was performed, but it failed to improve the condition, and the patient died on January 15. We described a case of non-Hodgkin's lymphoma complicated by hepatic dysfunction, probably induced by CPM, in an elderly patient who died to hepatic failure.     

Non-Hodgkin's lymphoma complicated by recurrent intractable generalized herpes zoster responsive to long-term acyclovir therapy]

A 34-year-old male with a history of chickenpox developed primary abdominal non-Hodgkin's lymphoma (diagnosed in August 1995). Treatment with cyclophosphamide, doxorubicin, vincristine, and prednisolone achieved a partial remission. In July 1996, the disease recurred, and the patient received chemotherapy with carboplatine, etoposide, mitoxantrone, and prednisolone, but no response was noted. Involvement of the central nervous system and meninges was diagnosed on September 12, 1997. Blast cells were detected in the peripheral blood on September 26. Based on these findings, he was diagnosed as having leukemia. On September 27, painless vesicles developed on the left gluteal region. On October 13, the patient was hospitalized because the vesicles had spread over his entire body. Pathologic examination of the roofs of the blisters showed masses of inclusion bodies. Based on this, a diagnosis of varicella-zoster infection was made. Treatment with acyclovir (750 mg/day) for seven days failed to form crusts. New vesicles developed after the drug was discontinued, but crusts formed after acyclovir therapy was resumed. He died of interstitial pneumonia on December 21. Autopsy could not be performed. Histopathologic examination of pulmonary tissue obtained by necropsy did not reveal the presence of inclusion bodies characteristic of herpes simplex or varicella-zoster infection. Varicella-zoster virus (VZV) antigen was negative by an immunochemical staining method using monoclonal antibodies against VZV. Continuous long-term administration of acyclovir has been reported to be effective for non-Hodgkin's lymphoma complicated by recurrent intractable herpes zoster.     

Long-lasting remission of primary hepatic lymphoma and hepatitis C virus infection achieved by the alpha-interferon treatment

Primary hepatic lymphoma is a rare but well-defined lymphoma entity that often pursues an aggressive clinical course. Most cases have been described in hepatitis C virus (HCV)-related chronic liver disease patients. Although anthracycline-based chemotherapy has been reported to be highly effective, the best therapeutic strategy has not been defined yet. The prognosis is dismal especially in patients treated with chemotherapy alone or when an advanced liver disease is present. Herein, we describe a case of primary hepatic large B-cell non-Hodgkin's lymphoma, in a patient with HCV chronic infection. After a minor response with eight cycles of CHOP chemotherapy, a complete and sustained remission was obtained with alpha-interferon at the daily dose of 3 MU. HCV-RNA clearance pace from the blood almost paralleled the response of the lymphoma and both diseases went in remission within 1 year of therapy. The possible place of alpha-Interferon in the treatment of primary hepatic lymphoma is discussed.     

Disseminated varicella-zoster virus infection without vesicles in a patient with malignant lymphoma]

A 76-year-old man was diagnosed as having malignant lymphoma (non-Hodgkin's lymphoma, diffuse medium cell-sized, B cell type). He was treated with CHOP therapy but with no response. In the terminal stage, he had continuous high temperature despite the administration of anti-bacterial and anti-fungal agents. Paralytic ileus, liver and pancreas dysfunction, and gastrointestinal bleeding developed. No skin eruptions occurred throughout the clinical course. He died on day 36 of treatment. Postmortem examination revealed foci of hemorrhagic necrosis containing many multinuclear giant-cells some of which with intranuclear inclusion bodies (Cowdry type A), in the liver, pancreas, gastrointestinal tract, bone marrow and other organs. Electron microscopy showed viral particles in the cytoplasm but not the nuclei of infected cells which were covered with a capsule, which was characteristic of varicella-zoster virus infection. Cells of the above organs were positive for immunohistochemical staining using antivaricella-zoster antibodies. The multiorgan failure seen in the terminal stage was considered to be due to disseminated varicella-zoster infection.     

Primary pleural non-Hodgkin's lymphoma without chronic pyothorax]

Most cases of primary pleural malignant lymphoma develop following chronic pyothorax. We report a case of primary pleural non-Hodgkin's lymphoma without chronic pyothorax. A 63-year-old woman was referred and admitted to our hospital with a right pleural effusion that was detected during a routine physical checkup. Her liver, spleen, and superficial lymph nodes were not palpable on physical examination. The massive right pleural effusion and a pleural mass were demonstrated on chest X-ray films and thoracic computed tomograms. Diffuse large B-cell non-Hodgkin's lymphoma was diagnosed by needle biopsy from the pleura, and the clinical stage was IE. Pleural effusion specimens contained no identifiable lymphoma cells, and examinations for Mycobacterium species were also negative. Human herpes virus 8 (HIV-8) DNA was detected in lymphocytes from the peripheral blood and pleural effusion. Epstein-Barr virus-encoded small RNAs and HHV-8 DNA were both negative in biopsied tissue from the pleural mass. Although a complete remission was achieved, the lymphoma relapsed about 8 months later. The patient is currently receiving salvage chemotherapy. Cases of primary pleural non-Hodgkin's lymphoma with massive pleural effusion that are not preceded by chronic pyothorax or Kaposi's sarcoma are very rare.     

Non-Hodgkin's lymphoma presenting as a primary tumor of the liver: presentation, diagnosis and outcome in eight patients

We report the diagnostic, prognostic and therapeutic features of non-Hodgkin's lymphoma in eight patients in whom the disease was seen as a primary tumor of the liver. This series illustrates the variety of situations in which lymphoma might be diagnosed: (a) abdominal pain and hepatomegaly (three cases), (b) incidental finding at evaluation of a patient with cirrhosis (two cases), (c) secondary neoplasm after treatment for Hodgkin's disease (one case) and (d) complication of AIDS (two cases). In most cases, clinical and/or radiological features were nonspecific. However, the combination of the following features must be considered as suggestive: occurrence of an apparently primary hepatic tumor in an immunocompromised patient, absence of the usual serum tumor markers and increased serum lactic dehydrogenase activity. The final diagnosis was based on histological examination of specimens obtained by ultrasonically guided liver biopsies or at surgery. All cases belonged to unfavorable histological subtypes. Immunohistochemical findings on paraffin-embedded sections demonstrated the B-lymphocyte lineage of the seven tumors available for study. In the three patients without coexisting disease, complete remission was obtained by surgery alone or combined with chemotherapy. In the two patients with coexisting cirrhosis, outcome was rapidly unfavorable, with death occurring less than 3 mo after diagnosis. Among the three immunocompromised patients, two experienced a rapid unfavorable outcome, and the remaining one was in complete remission after surgery and chemotherapy. In conclusion, primary non-Hodgkin's lymphoma of the liver arising in patients without coexisting disease has a slow progression and might be successfully treated by surgery.(ABSTRACT TRUNCATED AT 250 WORDS)     

Acute Liver Injury Due to T-cell Infiltration into the Liver as an Initial Clinical Finding of Adult T-cell Leukemia/Lymphoma

Acute liver injury (ALI) has been rarely reported as a clinical finding of adult T-cell leukemia/lymphoma (ATLL). A 74-year-old Japanese female patient who was histologically diagnosed as having autoimmune hepatitis (AIH) one year earlier, showed elevations in her aminotransferase and total bilirubin levels, and this was considered to be an exacerbation of AIH. Liver biopsy revealed interface hepatitis. Because atypical lymphocytes and human T-cell leukemia virus 1 immunoglobulin G antibody were positive, the patient was diagnosed to have ATLL. The biopsy revealed CD4+ and CD8+, but not CD20+ lymphocytes. Thus, the ALI in the patient was due to T-cell infiltration into the liver, and not due to an exacerbation of AIH.     

Human Herpes Virus 8-Negative Primary Effusion Lymphoma in a Patient With a Ventriculoperitoneal Shunt Tube

A 60-year-old woman was referred to our hospital in 1996 due to an abdominal distension in the right lower quadrant. She had undergone a partial resection of a cholesteatoma at the right temporal lobe of the cerebrum 30 years previously, and a ventriculoperitoneal shunt (VPS) tube had been placed with drainage into the right lower peritoneal cavity. The patient developed paralytic ileus in December 1966, and ultrasound and computed tomography of the abdomen revealed a cystic mass in the right lower quadrant without lymphadenopathies or masses. Cytologic examinations of the fluid in the cystic mass revealed signs of malignant lymphoma. After the resection of the cystic mass, lymphoma cells were detected in the fluid, but the wall of the cyst consisted of only fibrous tissues. Results of immunophenotypic analysis of the lymphoma cells by immunocytochemistry or flow cytometry were positive for CD19, CD20, CD22, CD45, and HLA-DR but negative for CD45RO, CD3, CD4, and CD8. The genome of human herpes virus (HHV)-8 was not detected in the lymphoma cells, but Epstein-Barr (EB) nuclear antigen 1 and EB virus (EBV)-encoded small nuclear RNAs were detected. Chromosome analysis by the G-banding method showed complicated abnormalities including der(8)t(2;8)(q31;q24), but Southern blotting analysis suggested that the c-myc oncogene did not participate in the lymphomagenesis. The patient's disease was diagnosed as HHV-8-negative primary effusion lymphoma (PEL). The long-standing inflammatory stimulation by a VPS tube might have contributed to the clonal evolution of EBV-infected lymphocytes. resulting in the development of PEL.     

Spontaneous Intestinal Perforation Due to Non-Hodgkin's Lymphoma: Evaluation of Eight Cases

Primary intestinal lymphoma with spontaneous perforation and after systemic chemotherapy is rare. The present study summarizes retrospectively the outcome of eight free intestinal perforated patients diagnosed with intestinal non-Hodgkin's lymphoma. Two patients had a history of systemic chemotherapy before perforation. The most common symptoms of the patients were abdominal pain, nausea, vomiting, weight loss, and fever. Sites of perforation were ileum in four, jejunum in two, cecum in one, and sigmoid colon in one patient. Synchronous lymphoma was present in three patients. The perforation was closed by primary closure in three patients. Resection/anastomosis was performed in four patients and sigmoid colostomy was performed in one patient. Three patients were lost due to leakage or septicemia. Intestinal lymphoma might be kept in mind as a cause in free intestinal perforations. Because of the high mortality rate early diagnosis and treatment are important to improve the prognosis of bowel perforation in patients with non-Hodgkin's lymphoma.     

Secondary pancreatic involvement by a diffuse large B-cell lymphoma presenting as acute pancreatitis

Diffuse large B-cell lymphoma is the most common type of non-Hodgkin's lymphoma. More than 50% of patients have some site of extra-nodal involvement at diagnosis, including the gastrointestinal tract and bone marrow. However, a diffuse large B-cell lymphoma presenting as acute pancreatitis is rare. A 57-year-old female presented with abdominal pain and matted lymph nodes in her axilla. She was admitted with a diagnosis of acute pancreatitis. Abdominal computed tomography (CT) scan showed diffusely enlarged pancreas due to infiltrative neoplasm and peripancreatic lymphadenopathy. Biopsy of the axillary mass revealed a large B-cell lymphoma. The patient was classified as stage Ⅳ, based on the Ann Arbor Classification, and as having a high-risk lymphoma, based on the International Prognostic Index. She was started on chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone). Within a week after chemotherapy, the patient’s abdominal pain resolved. Follow-up CT scan of the abdomen revealed a marked decrease in the size of the pancreas and peripancreatic lymphadenopathy. A literature search revealed only seven cases of primary involvement of the pancreas in B-cell lymphoma presenting as acute pancreatitis. However, only one case of secondary pancreatic involvement by B-cell lymphoma presenting as acute pancreatitis has been published. Our case appears to be the second report of such a manifestation. Both cases responded well to chemotherapy.     

Non-Hodgkin's lymphoma in two married couples

Couple 1: A 74-year-old woman was diagnosed as having diffuse large B-cell lymphoma (DLBL) by left axillary lymph node biopsy. About 6 months later, DLBL was also diagnosed in her 79-year-old husband by right submandibular lymph node biopsy. Although the wife achieved partial remission with chemotherapy, she died due to disease progression. The husband's disease was chemotherapy-resistant, and he died of renal failure. Couple 2: An 86-year-old man was diagnosed as having DLBL by left axillary lymph node biopsy. About 4 years later, DLBL was also diagnosed in his 86-year-old wife by left axillary lymph node biopsy. Both the husband and the wife received chemotherapy. The husband is currently alive in complete remission, and although the wife achieved partial remission, she died due to disease progression. In both of these couples, it was considered unlikely that Epstein-Barr virus or human T-cell lymphotropic virus type I was related to the development of non-Hodgkin's lymphoma, and no environmental factors were confirmed to be involved. It is postulated that other unknown factors or agents may be associated with the development of lymphoma in married couples.     

Fulminant liver failure associated with T-cell non-Hodgkin's lymphoma and hepatitis C virus: a case report

Hematological malignancies can affect the liver, without producing severe hepatic involvement. We report the case of a 57-year-old man with hepatitis C virus infection and mild chronic hepatitis without antiviral treatment, who developed an aggressive T-cell non-Hodgkin's lymphoma confirmed by histological studies including liver, lymph nodes and bone marrow. The patient developed massive hepatic infiltration and acute liver failure. Rescue chemotherapy was administered but the patient died soon after with severe lactic acidosis. The immunopathological features of this association and the few reports of cases presenting with acute liver failure are reviewed.     

Primary gastric T-cell lymphoma not associated with human T-lymphotropic virus type I: a case report and review of the literature

Primary gastric T-cell lymphoma (PGTL) not associated with human T-lymphotropic virus type I (HTLV-I) is extremely rare and such a case is reported herein. The patient was a 58-year-old Japanese male presenting with submucosal tumor of the stomach identified on endoscopic examination. The lesion was diagnosed as non-Hodgkin's lymphoma by endoscopic biopsy and classified as peripheral T-cell lymphoma, unspecified, due to clonal rearrangement of the T-cell receptor beta (TCR) gene and expression of TCR beta protein in the absence of B-cell genotypes and phenotypes. Unlike previously reported cases of HTLV-I-unassociated PGTL, lymphoma in the current case was characterized histologically as "low grade" and phenotypically as CD4+, TIA-1+, granzyme B+, and CD103-. The lymphoma responded well to chemotherapy and radiation, and the patient was well with no detectable disease 10 months after initiation of therapy. A review of patients with PGTL in the literature revealed a few long-term survivors, and the investigation of therapeutic strategies for PGTL is, therefore, necessary.     

Non-Hodgkin's lymphoma localized at the right ocular medial rectal muscle

This paper reports a rare case of extranodal non-Hodgkin's lymphoma localized at the ocular muscle, which achieved a long-term remission by combination chemotherapy and irradiation. A 41-year-old man visited the ophthalmology department of Toho University Hospital in December, 1982 because of right conjunctival injection and right exophthalmos which were first manifested in June. The patient was diagnosed as having non-Hodgkin's lymphoma (diffuse medium-sized cell type) by biopsy of the right medial rectal muscle, and was referred to our department on January 17, 1983. A series of examinations revealed that the disease had no invasion into any lymph nodes and other organs. After treatment with two courses of C-MOPP regimen and two courses of ACOP regimen, the patient achieved complete remission and was discharged on July 30. Subsequently he completed two courses of CHOP regimen followed by irradiation (46 Gy). Seven years and three months after the initiation of chemotherapy, the patient has been in a complete remission, requiring no treatment.     

Double B-cell malignancies with simultaneous onset

We encountered a case of a 59-year-old female who simultaneously contracted a non-Hodgkin lymphoma (NHL) and a plasma cell neoplasm. The patient consulted her physician about her abdominal tumor and anemia in March 1999. She was diagnosed as having NHL (follicular center lymphoma, grade I, stage IIA) after an open tumor biopsy, and treated by cycles of CHOP chemotherapy which resulted in complete remission. However, the patient's abdominal tumor appeared again in March 2000 and she was hospitalized at the Ehime University Hospital. A tumor biopsy was performed laparoscopically at that time. Follicular lymphoma (with positive LCA, L-26, and bcl-2 immuno-staining) with the development of retroperitoneal fibrosis was diagnosed again. When a bone marrow puncture was performed because of a condition of monoclonal gammopathy which had continued for two years, a smoldering myeloma was additionally diagnosed. This diagnosis was made after the presence of IgG-lambda M protein when the marrow showed an increase in the number of plasma cells. In a Southern blot analysis which studied the abdominal tumor and the bone marrow cells, each B-cell tumor had a different IgH gene rearrangement pattern. Therefore, this case was diagnosed as an example of the simultaneous existence of two different B-cell tumors. Double cancers in hematological malignancies are very rare and this was thought to be an interesting case.     

Radiation-induced stricture of the papilla and the common bile duct: successful treatment with balloon dilation

Radiation-induced lesions of the bile ducts rarely occur and may be difficult to manage. We report the case of a 59-year old woman who developed radiation-induced stenosis of the papilla and the common bile duct 25 years after abdominal radiation therapy for abdominal non-Hodgkin's lymphoma. She presented with recurrent cholangitis and chronic cholestasis. Endoscopic results showed dilation of the intrahepatic bile ducts, radiation-induced inflammation and narrowing of the antrum and the duodenum, and stricture of the papilla and the last few millimeters of the common bile duct. The patient was treated with endoscopic balloon dilation. Forty-two months after endoscopic dilation, the patient remained asymptomatic with normal liver tests and no biliary dilation at ultrasound.     

CD30/Ki-1-positive anaplastic large cell lymphoma preceded by Hodgkin's disease.

A 59-year-old man was initially diagnosed as having Hodgkin's disease, nodular sclerosis type, and complete remission was achieved after combination chemotherapy. One year later, he developed a high fever and recurrence of the Hodgkin's disease was diagnosed. Salvage chemotherapy was ineffective, and the patient died. Autopsy specimens showed infiltration of lymphoma cells into multiple organs. Lymph nodes showed characteristics of non-Hodgkin's lymphoma, with expansion of anaplastic large cells; this differed from the histological features at initial diagnosis. Immunohistochemical staining was positive for CD30/Ki-1, but negative for CD15 (LeuM1). These findings were compatible with Ki-1 lymphoma, suggesting that this may be a case of CD30/Ki-1 lymphoma preceded by Hodgkin's disease and that a certain proportion of Ki-1 lymphomas and Hodgkin's disease may share the same cellular origin.