A rare case of enteropathy-associated T-cell lymphoma presenting as acute renal failure

Enteropathy-associated T-cell lymphoma (EATCL) is a high grade, pleomorphic peripheral T-cell lymphoma usually with cytotoxic phenotypes. We describe a first case of patient with EATCL that is remarkable for its fulminant course and invasion of both kidneys manifested as acute renal failure. The patient was a 23 year old woman with a long history of celiac disease. She was presented with acute renal failure and enlarged mono-nuclear infiltrated kidneys. Diagnosis of tubuloi-nterstitial nephritis and polyserositis was confirmed with consecutive pulse doses of steroid therapy. After reco-very, she had disseminated disease two months later. Magnetic resonance imaging showed thickened intestine wall, extremely augmented kidneys, enlarged intra-abdominal lymph nodes with extra-luminal compression of common bile duct. Laparotomy with mesenterial adipous tissue and lymph glands biopsy was done. Consecutive pathophysiological and immunohistochemical analyses confirmed the diagnosis of EATCL: CD45RO , CD43 , CD3 . The revision of renal pathophysiology sub-stanti-ated the diagnosis. The patient received chemotherapy, but unfortunately she died manifesting signs of pulmonary embolism caused by tumor cells.     

Acute liver failure complicating jejunojejunal intussusception presentation in a gastric bypass patient

Over 200 000 weight loss procedures are performed annually in the United States. Physicians must there-fore be cognizant of the unique array of complications associated with these procedures. We describe a case of jejunojejunal intussusception in a gastric bypass patient who presented with acute liver failure (ALF) due to acetaminophen (APAP) toxicity. Our patient is a 29 year-old female who had undergone Roux-en-Y gastric bypass surgery seven years prior. She was evaluated in the emergency department for confusion. Her family reported a 3-wk history of progressive abdominal pain and vomiting, for which she had ingested 40 acetamin-ophen/oxycodone tablets over the past 2 d. Physical examination showed icteric sclerae, a distended abdomen, and grade Ⅰencephalopathy. She fulfilled the criteria for ALF and was listed for liver transplantation. Abdominal computed tomography scan revealed a je-junojejunal intussusception. She under went emergent exploratory laparotomy and resection of the infarcted intussusceptum and the previous jejunojejunostomy. She had rapid clinical improvement, with decreasing liver enzymes and improved hepatic synthetic function. She had complete resolution of coagulopathy and encephalopathy, and was removed from the liver transplant list. She was discharged home 20 d after hospitalization with normal liver tests. This case demonstrates that acute abdominal catastrophes can potentiate liver injury in the setting of acetaminophen toxicity. Encephalopathy may obscure history and physical exam findings. This case also exemplifies the pitfalls in the management of the bariatric surgery patient and the importance of multispecialty collaboration in patients presenting with organ failure.     

Anabolic steroid-induced cardiomyopathy underlying acute liver failure in a young bodybuilder

Heart failure may lead to subclinical circulatory disturbances and remain an unrecognized cause of ischemic liver injury. We present the case of a previously healthy 40-year-old bodybuilder, referred to our Intensive-Care Unit of Hepatology for treatment of severe acute liver failure, with the suspicion of toxic hepatitis associated with anabolic steroid abuse. Despite the absence of symptoms and signs of congestive heart failure at admission, an anabolic steroid-induced dilated cardiomyopathy with a large thrombus in both ventricles was found to be the underlying cause of the liver injury. Treatment for the initially unrecognized heart failure rapidly restored liver function to normal. To our knowledge, this is the first reported case of severe acute liver failure due to an unrecognized anabolic steroid-induced cardiomyopathy. Awareness of this unique presentation will allow for prompt treatment of this potentially fatal cause of liver failure.     

A histologically proven case of progressive liver sarcoidosis with variceal rupture

Sarcoidosis is a chronic multi-systemic granulomatous disease,and liver involvement frequently occurs.in most cases,no evidence of liver dysfunction is ob-served,and portal hypertension due to sarcoid liver diseases is a rareoccurrence.Moreover,no case of liver sarcoidosis has ever been reported with confirma-tion of the disease progression.Herein we describe a patient having hepatic sarcoidosis with severe portal hypertension and liver dysfunction.The diagnosis was histologically confirmed from granulomatous status to established liver cirrhosis over 10 years.A 46-year-old woman developed massive hematemesis due to the rupture of gastric cardial varices.She underwent emer-gency endoscopic injection sclerotherapy,and clear evi-dence of chronic hepatic failure.Twelve years ago,she was diagnosed as having sarcoidosis with respiratoryclinicalsymptoms.Liver biopsy revealed asymptomatic incidental granulomas without fibrosis development.After a couple of years,features of liver dysfunction were manifest and progressed.Ten years after the first biopsy,a second liver biopsy was performed,and well established dense fibrosis was revealed.Although significant liver dysfunction with portal hypertension is rarely seen in sarcoidosis,this case indicates that we have to consider the possibility that sarcoidosis may cause end-stage liver cirrhosis.     

Parvovirus B19 induced hepatic failure in an adult requiring liver transplantation

Parvovirus B19 induced acute hepatitis and hepatic failure have been previously reported, mainly in children. Very few cases of parvovirus induced hepatic failure have been reported in adults and fewer still have required liver transplantation. We report the case of a 55-year-old immunocompetent woman who developed fulminant hepatic failure after acute infection with Parvovirus B19 who subsequently underwent orthotopic liver transplantation. This is believed to be the first reported case in the literature in which an adult patient with fulminant hepatic failure associated with acute parvovirus B19 infection and without hematologic abnormalities has been identified prior to undergoing liver transplantation. This case suggests that Parvovirus B19 induced liver disease can affect adults, can occur in the absence of hematologic abnormalities and can be severe enough to require liver transplantation.     

Endoscopic biopsy： Duodenal ulcer penetrating into liver

TO THE EDITOR We have read with interest the recent report by E Kayacetin and S Kayacetin of Gastric ulcer penetrating to liver diagnosed by endoscopic biopsy[1] since we diagnosed the duodenal ulcer which penetrated into liver similarly. This is a rather unusual case because of the fifth case in the literature and responding to medical therapy.     

Endoscopic biopsy:Duodenal ulcer penetrating into liver

TO THE EDITOR We have read with interest the recent report by E Kayacetin and S Kayacetin of "Gastric ulcer penetrating to liver diagnosed by endoscopic biopsy since we diagnosed the duodenal ulcer which penetrated into liver similarly. This is a rather unusual case because of the fifth case in the literature and responding to medical therapy.     

Multi-organ failure with atypical liver granulomas following intravesical Bacillus Calmette-Guerin instillation

Bacillus Calmette-Guerin(BCG) intravesical instillation has been adopted in the treatment of patients with superficial bladder cancer.BCG-induced disseminated infection,though rare,has been associated with the histological finding of epithelioid granulomas in different organs,including the liver.We report the case of an adult patient with multi-organ failure,who developed sepsis,acute respiratory failure and acute hepatic failure with encephalopathy whose liver biopsy confirmed the presence of atypical,granulomatous-like lesions.Recovery was observed only after empirical therapy for Mycobacterium bovis with isoniazid,rifampicin,ethambutol and steroids was introduced.This case highlights the importance of a thorough patient assessment in order to exclude other more common causes of hepatic granulomas and to confirm diagnosis.Histological findings may be non-specific when the liver is involved in BCGinduced disseminated infection.     

Vanishing bile duct syndrome in Hodgkin's lymphoma: A case report and literature review

Vanishing bile duct syndrome(VBDS) has been described in different pathologic conditions including infection, ischemia, adverse drug reactions, autoimmune diseases, allograft rejection, and humoral factors associated with malignancy. It is an acquired condition characterized by progressive destruction and loss of the intra-hepatic bile ducts leading to cholestasis. Prognosis is variable and partially dependent upon the etiology of bile duct injury. Irreversible bile duct loss leads to significant ductopenia, biliary cirrhosis, liver failure, and death. If biliary epithelial regeneration occurs, clinical recovery may occur over a period of months to years. VBDS has been described in a number of cases of patients with Hodgkin's lymphoma(HL) where it is thought to be a paraneoplastic phenomenon. This case describes a 25-year-old man found on liver biopsy to have VBDS. Given poor response to medical treatment, the patient underwent transplant evaluation at that time and was found to have classical stage ⅡB HL. Early recognition of this underlying cause or association of VBDS, including laboratory screening, and physical exam for lymphadenopathy are paramount to identifying potential underlying VBDS-associated malignancy. Here we review the literature of HL-associated VBDS and report a case of diagnosed HL with biopsy proven VBDS.     

Malignant lymphoma of spleen presenting as acute pancreatitis： A case report

This is a case report of a patient who presented with acute pancreatitis without the common causes. A pancreatic biopsy revealed large B cell lymphoma. Spleen lymphoma with pancreatic involvement inducing acute pancreatitis, which is a rare disorder, was diagnosed. Here we also review the few similar cases reported in the literature.     

T-cell lymphoma associated with natural killer-like T-cell reaction

We report a case of T-cell lymphoma showing in the peripheral blood (PB) exclusively T-lymphocytes with suppresser T-cell preponderance and a high percentage of natural killer (NK) marker positive cells by flow cytometry. A T-cell receptor (TCR) gene analysis of the PB leukocytes demonstrated rearrangements of TCRα, TCRβ, and TCRγ genes. Therefore, the phenotype and genotype appeared to be consistent with an NK-like T-cell leukemia/lymphoma. However, when the PB lymphocytes were separated by size, it was found that 80% of NK marker positive cells were in the smaller cell population, while the neoplastic cells were in the large cell gate. A diagnosis of T-cell lymphoma with reactive NK-like T-cells was finally confirmed by demonstrating the presence of both large atypical lymphoid cells and large granular lymphocytes (LGL) on PB smears. Although immunoperoxidase stain of bone marrow and colon showed positive T-cell markers in the tumor cell population, cytoplasmic granules could not be identified in tissue sections and, thus, a distinction between T-cell lymphoma and NK-like T-cell lymphoma could not be made by light microscopy until NK markers were studied. CD57 was demonstrated immunohistochemically in small lymphocytes but not in the large tumor cells in the colon. Electron microscopy, however, demonstrated LGL reaction to the lymphoma cells in the colonic biopsy. NK-like T-cell lymphoma usually carries a poorer prognosis than peripheral T-cell lymphoma, thus the distinction of these neoplasms is important. This study emphasizes that T-cell lymphoma may cause an LGL reaction or proliferation. If the lymphoma cells were of the same size as LGL, flow cytometric studies may have misled the diagnosis to NK-like T-cell-lymphoma. Am. J. Hematol. 57:331–337, 1998. © 1998 Wiley-Liss, Inc.     

Enteropathy-associated intestinal t-cell lymphoma without celiac disease

Type II enteropathy-associated T-cell lymphoma (EATL) is an uncommon intestinal lymphoma. We report the case of a 73-year-old man with diarrhea and weight loss. Duodenal biopsy showed atrophy and infiltration of irregular lymphocytes. Immunohistochemistry was positive for CD3, CD8, and CD56 with monoclonal TCR rearrangement. The HLA-DQ genotype was DQ5/DQ9. The Epstein-Barr virus RNA test was negative. Before specific chemotherapy could be administered, the patient was admitted to hospital for a respiratory infection and died from a cause unrelated to his lymphoma. The differential diagnosis of CD56-positive lymphoproliferative processes include type II EATL, primary T-cell/natural killer-cell intestinal lymphoma and hepatosplenic T-cell lymphoma. The patient had CD8 y CD56+ markers that allowed type I EATL to be excluded. The HLA-DQ genotype did not correspond to celiac disease and the biopsy showed proliferation of lymphocytes with atypia. The primary intestinal T-cell/natural killer-cell lymphoma was characterized mainly by the absence of CD8 and monoclonal reassortment of the TCR present in this case.     

Natural killer-like T-cell lymphoma appearing in the duodenum with recurrence in a variety of extranodal organs]

A 48-year-old man was admitted to Keio University Hospital in April 1995 with complaints of right abdominal pain and weight loss. Hypotonic duodenography showed a mass located in the 3rd portion of the duodenum. Endoscopic biopsy specimens disclosed diffuse large cell non-Hodgkin's lymphoma of the duodenum. The patient was initially treated with 2 courses of CHOP, albeit with no response. A pancreatoduodenectomy and radiotherapy yielded a complete remission. A year later, lymphoma recurred in the right mandible salivary gland, and a second complete remission was obtained after 6 courses of CHOP and radiation. However lymphomas also recurred in the intestine, and lungs, and the patient died of disease progression 38 months after diagnosis. Lymphoma cells were surface CD3 and CD56 positive. An examination of resected intestinal tissues disclosed lymphoma cells morphologically resemble large granular lymphocytes with rearranged TcR genes. These findings indicated the diagnosis of natural killer-like (NK-like) T-cell lymphoma. Compared with previously reported cases of NK-like T-cell lymphoma, this case was noteworthy for an unusual clinical course characterized by initial appearance in the duodenum, recurrence in a variety of extranodal organs, and the relatively long-term survival of the patient.     

Primary hepatic peripheral T-cell lymphoma associated with Epstein-Barr viral infection

Primary hepatic peripheral T-cell lymphoma(H-PTCL) is one of the rarest forms of non-Hodgkin lymphoma. We report a patient who presented with worsening jaundice, abdominal pain, and vomiting. Laboratory values were significant for elevated total bilirubin, alkaline phosphatase, and liver aminotransferases. Following a liver biopsy, histopathology revealed several large dense clusters of atypical T-lymphocytes which were CD2+, CD3+, CD5+, CD7-, CD4+, CD8-, CD56-, CD57-, CD30+ by immunohistochemistry. The proliferation index was approximately 70% by labeling for ki67/mib1. The above histological profile was consistent with peripheral T-cell lymphoma of the liver. Epstein-Barr viral serology indicated a remote infection, a likely risk factor for PTCL. Bone marrow biopsy was negative for malignancy, further supporting hepatic origin.     

Acute liver failure secondary to acute antibody mediated rejection after compatible liver transplant: A case report

BACKGROUNDThe liver has traditionally been regarded as resistant to antibody-mediated rejection (AMR). AMR in liver transplants is a field in its infancy compared to kidney and lung transplants. In our case we present a patient with alpha-1-antitrypsin disease who underwent ABO compatible liver transplant complicated by acute liver failure (ALF) with evidence of antibody mediated rejection on allograft biopsy and elevated serum donor-specific antibodies (DSA). This case highlights the need for further investigations and heightened awareness for timely diagnosis.CASE SUMMARYA 56 year-old woman with alpha-1-antitrypsin disease underwent ABO compatible liver transplant from a deceased donor. The recipient MELD at the time of transplant was 28. The flow cytometric crossmatches were noted to be positive for T and B lymphocytes. The patient had an uneventful recovery postoperatively. Starting on postoperative day 5 the patient developed fevers, elevated liver function tests, distributive shock, renal failure, and hepatic encephalopathy. She went into ALF with evidence of antibody mediated rejection with portal inflammation, bile duct injury, endothelitis, and extensive centrizonal necrosis, and C4d staining on allograft biopsy and elevated DSA. Despite various interventions including plasmapheresis and immunomodulating therapy, she continued to deteriorate. She was relisted and successfully underwent liver retransplantation.CONCLUSIONThis very rare case highlights AMR as the cause of ALF following liver transplant requiring retransplantation.     

Hemophagocytic syndrome as a presenting sign of transformation of smoldering to acute adult T-cell leukemia/lymphoma: efficacy of anti-retroviral and interferon therapy

A 55-year-old Caribbean woman with a 6-year history of smoldering adult T-cell leukemia/lymphoma presented with clinical and biological symptoms of hemophagocytic syndrome. An extensive search for infectious diseases was negative. A lymph node biopsy showing large T-cell lymphoma (CD4-, CD25+) and findings of high LDH count and severe lymphocytosis led to the diagnosis of acute adult T-cell leukemia/lymphoma. Anti-retroviral therapy combining zidovudine, lamivudine, and interferon-alpha was started, resulting in rapid control of both hemophagocytic syndrome and symptoms of acute adult T-cell leukemia/lymphoma. Thus, we propose that adult T-cell leukemia/lymphoma must be added to the spectrum of etiologies of hemophagocytic syndrome.     

Preferential infiltration of liver sinusoids in acute lymphoblastic leukemia

We report a case of acute lymphoblastic leukemia (ALL) presenting as severe jaundice. The patient, a 59-year-old man, was found to have abnormal liver function, including an elevated total bilirubin level (13.5 mg/dl) with hepatosplenomegaly, but no detectable lymphadenopathy. A liver biopsy and bone marrow examination revealed a lymphoid neoplasm. Pathologic features included invasion of an abnormal clone into the sinusoidal region of the liver, diffuse bone marrow involvement (41.6% of all nucleated cells) and splenomegaly. Small numbers of malignant cells were also detected in the peripheral blood. B-cell markers, such as terminal deoxynucleotidyl transferase (TdT), CD10, CD19, CD20 and HLA-DR were positive, and CD2, CD3, CD4, CD5, CD7, CD8, kappa, lambda, cytoplasmic mu and myeloperoxidase were negative. Cytogenetic analysis detected hyperdiploidy. In this case, a dose-attenuated CHOP regimen attained complete remission. To date, preferential infiltration to liver sinusoids has been noted in hepatosplenic gamma/delta T-cell lymphoma, other NK/T-cell malignancies, and some cases of hairy cell leukemia. Severe jaundice due to preferential infiltration of leukemic cells into liver sinusoids is rather uncommon as a presenting feature of ALL.     

Successful Treatment of Nasal T-Cell Lymphoma With a Combination of Local Irradiation and High-Dose Chemotherapy

Nasal natural killer (NK)/T-cell lymphoma is characterized by an aggressive clinical course and poor prognosis. The term "NK/T-cell" lymphoma includes both the NK-cell type and the T-cell type, which are classified by immunophenotyping and according to T-cell receptor (TCR) rearrangement. In addition, CD56+ T-cell lymphoma is defined as NK-like T-cell lymphoma. This report concerns a 54-year-old woman with nasal T-cell lymphoma. Its phenotype showed pure T-cell type with CD3+, CD56-, and TCR+ accompanied by Epstein-Barr virus infection. Although the lesions were localized in the nasal mucosa and facial skin (stage IE), local irradiation could not achieve complete remission (CR). We then administered 5 courses of CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisolone) regimen followed by high-dose chemotherapy with an autologous peripheral blood stem cell transplantation. This therapy resulted in CR. Our results suggest that this lymphoma subtype may be cured by means of intensive treatment soon after diagnosis.     

CD4- and CD56-positive T-cell line, MTA, established from natural killer-like T-cell leukemia/lymphoma

We have established a T-cell line, MTA, from the peripheral blasts of a patient with natural killer (NK)-like T-cell leukemia/lymphoma. The MTA cell displays a T-cell and NK-cell phenotype (CD2+, CD3+, CD4+, CD56+) identical to freshly isolated leukemic blasts from the patient. This cell line shows clonal T-cell receptor rearrangement and a distinguishable character by light microscopy with May-Grünwald Giemsa staining. G-banding analysis showed that the MTA cells had a karyotype of 94(4N), XXXX, add (1) (p36), del (5) (q14q23), add (17) (p11), add (19) (q13). However, unlike NK malignancy, we could not show a direct pathogenic role for Epstein-Barr virus (EBV) with EBV-encoded small RNA and polymerase chain reaction analysis. The MTA cell line is a novel cell line with which to study NK-like T-cell ontogenecity.     

Acute liver failure secondary to hepatic infiltration: a single centre experience of 18 cases

Background—Acute liver failure (ALF) secondary tomalignant infiltration of the liver is rare and is diagnosed often only after death.
Aims—To determine diagnostic factors andparticular clinical patterns of illness.
Methods—Review of case notes from all patientswith ALF secondary to hepatic infiltration admitted to this unit overan 18year period (1978-1995).
Results—From a total of 4020 admissions, 18 patients were identified with ALF attributable to hepatic infiltration.Mean age was 40.7 years. Aetiology was non-Hodgkin's lymphoma in ninepatients, Hodgkin's disease in three, infiltrative metastaticcarcinoma in four, and haemophagocytosis with no precipitant cause intwo cases. Prodromal symptoms were non-specific, but occurred at least two to four weeks before onset of ALF, making the presence of suchsymptoms of value in differential diagnosis of the cause of ALF.Clinical examination and investigations were unhelpful indistinguishing these cases from more usual causes of ALF. Usually, theclinical course was of rapid deterioration and death from multiorganfailure, and only one patient survived. Diagnosis was made during lifein 15 patients. Histology showed evidence of widespread hepatocellularnecrosis, with diffuse infiltration by tumour cells rather than focalcellular aggregation.
Conclusions—Only with accurate histologicaldiagnosis from liver biopsy and institution of specific therapy earlyin the management of such patients will the best chance of recovery beachieved. In every case of ALF with prodromal symptoms or abnormalimaging, hepatic histology should be obtained by liver biopsy as soonas possible to diagnose infiltrative hepatic disease.

Keywords:acute liver failure; hepatic infiltration; diagnosis; histology; prognosis