Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023890 (cirrhosis)
 42,195 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Patients with hepatitis C virus (HCV) infection develop a number of hematologic disorders, with benign and malignant B-cell proliferations being the most common. HCV-infected patients are also prone to developing peripheral cytopenias, the etiologies of which are multifactorial and include hypersplenism and/or antiviral medications. Some of these patients may undergo bone marrow biopsy but no study has systematically recorded the bone marrow findings in this patient group. Here, we report on the range of bone marrow findings in 47 adult HCV-infected patients. These patients, who lacked concurrent human immunodefiency virus (HIV) infection, most commonly presented for a bone marrow biopsy due to abnormal peripheral cell counts. The bone marrow biopsies displayed a range of findings. Dyserythropoiesis, present in 19% of the cases, was the most common finding. Patients with pancytopenia(n = 6), as defined by current World Health Organization standards, were the most likely to have bone marrow abnormalities; two pancytopenic patients had acute myeloid leukemia, and one patient had a primary myelodysplastic syndrome. There was no correlation in bone marrow findings and antiviral medications, MELD score, cirrhosis or splenomegaly, suggesting that the degree of bone marrow dysfunction is independent of stage of HCV. The results of this study suggest that bone marrow biopsy in HCV-infected patients, even those with features of hypersplenism and/or documented antiviral therapy, can be a valid test for hematologic evaluation, especially for patients with severe pancytopenia and/or sudden alterations in peripheral cell counts.
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PMID:Bone marrow biopsy in patients with hepatitis C virus infection: spectrum of findings and diagnostic utility. 2009 34

Choledochal cyst causes liver fibrosis, the extent of which varies with each case. Liver damage seldom progresses to cirrhosis, but when it does, it is generally irreversible. We report an infantile case of liver cirrhosis associated with choledochal cyst in which complete clinical resolution was achieved by surgery. Pancytopenia caused by splenomegaly, massive ascites, hypoalbuminemia, and coagulation abnormality that were observed during the early postoperative period had disappeared within 4 months after surgery. Needle liver biopsy performed at 1 year after surgery revealed marked improvement in liver fibrosis.
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PMID:Histopathologic improvement in biliary cirrhosis after definitive surgery for choledochal cyst. 2043 7

Dyskeratosis Congenita (DKC) is a rare progressive bone marrow disorder associated with multi-systemic involvement. It is also characterized by triad of abnormal skin pigmentation, nail dystrophy and mucosal leukoplakia. Liver cirrhosis and portal hypertension are said to be uncommon among these patients. We hereby report a case of an adult male who presented with pancytopenia, abnormal skin pigmentation, nail dystrophy and mucosal leukoplakia. Skin biopsies along with clinical features confirmed the case. Imaging studies were reported as suggestive of and portal hypertension. Liver biopsy done but non-conclusive. Patient's one son and one daughter also had similar skin pigmentation.
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PMID:Dyskeratosis congenita with portal hypertension of unknown etiology. 2175 68

Portopulmonary hypertension (POPH) is a not infrequent but serious complication of liver cirrhosis. Continuous intravenous epoprostenol infusion is a treatment option for this condition. Progressive splenomegaly with pancytopenia (hypersplenism) is associated with epoprostenol use in POPH. After recognizing a case of epoprostenol-induced hypersplenism that resolved upon stopping the drug, the authors retrospectively reviewed all patients treated with epoprostenol at the center for both POPH and pulmonary hypertension due to other causes. Five of 11 patients with POPH developed hypersplenism secondary to epoprostenol. In 1 patient, and possibly in a second, the hypersplenism resolved upon discontinuation of epoprostenol. None of 9 patients with pulmonary hypertension due to other causes developed splenomegaly. This report confirms hypersplenism as a complication of epoprostenol therapy for POPH. Furthermore, the authors demonstrate for the first time that hypersplenism may be reversed by stopping the medication and propose a mechanism for this phenomenon.
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PMID:Epoprostenol-induced hypersplenism in portopulmonary hypertension. 2222 13

We recently re-examined a case of Visceral Leishmaniasis, in a 36-year-old caucasian immune-competent men with an unusual clinical presentation. Together with symptoms and signs of a severe acute liver involvement, he presented weight loss, huge spleen enlargement, pancytopenia and increased ?-globulin serum level with a high polyclonal peak. He had no fever, but over-abundant night sweats were frequent. The patient was considered to have liver cirrhosis, and the diagnosis of visceral leishmaniosis was made with a year's delay. From this case report we may learn that, despite an unusual clinical presentation, the diagnosis of visceral leishmaniasis should not be excluded when other characteristic signs and symptoms and laboratory abnormalities are present.
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PMID:Acute liver failure: a rare clinical presentation of visceral leishmaniasis. 2237 60

Organ transplant recipients are at an increased risk for subsequent malignancies including hematologic malignancies. The development of acute myeloid leukemia (AML) after solid organ transplantation is a rare but well-documented event. It is thought to be a consequence of immune dysregulation secondary to the use of immunosuppressive agents. Herein, we present the management of a liver transplantation recipient who presented with AML and comprehensively review the relevant literature. A 59-year-old male patient presented with fever and cough eight years after an orthotopic liver transplantation for cirrhosis and hepatocellular carcinoma. He received methylprednisolone and mycofenolate mofetil (MMF) followed by tacrolimus and rapamycin as immunosuppression. Upon admission to our hospital, his peripheral blood demonstrated 34% blasts and pancytopenia. A bone marrow biopsy confirmed the diagnosis of myelodysplastic syndrome (MDS) in transformation to AML. He was treated with induction chemotherapy and his sirolimus was continued but he expired four weeks after from refractory disease. No specific guidelines exist for the treatment of AML in solid organ transplant recipients. Treatment should be individualized and concurrent use of chemotherapeutic and immunosuppressive agents should be carefully balanced.
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PMID:Acute myeloid leukemia following solid organ transplantation: case report and comprehensive review. 2266 74

The behavior of littoral cell neoplasms ranges from benign (littoral cell angioma, LCA) to highly malignant (angiosarcoma). Two unusual cases of low-grade metastatic littoral cell angiosarcoma (LCAS) have been reported with late recurrence and bulky metastases. We present the third case of this rare neoplasm in a 38-year-old man with cirrhosis and a large splenic artery aneurysm, without extrasplenic masses. The spleen showed nodules resembling LCA, immunoreactive for CD31, factor VIII, CD68, and CD163 but not CD8 or CD34. Also present were solid areas of immunophenotypically identical bland spindle cells, although lighter CD31 immunostaining distinguished them from LCA-like angiomatous channels. Similar cells diffusely infiltrated the cirrhotic liver. After splenectomy, pancytopenia resolved, and he is asymptomatic 19 months later. Low-grade LCAS is a previously unreported cause of cirrhosis and may metastasize without forming masses. In cases of LCA, CD31 immunohistochemistry may facilitate detection of LCAS and indicate metastatic potential.
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PMID:Low-grade, metastasizing splenic littoral cell angiosarcoma presenting with hepatic cirrhosis and splenic artery aneurysm. 2342 63

The response rate and overall survival after sorafenib administration in patients with advanced hepatocellular carcinoma are unsatisfactory. We herein present the case of a 65-year-old man with multiple lung metastases of hepatocellular carcinoma. Because the patient had liver cirrhosis of Child-Pugh B accompanied by pancytopenia, sorafenib administration was initiated at a dose of 400 mg daily. Although he received sorafenib for only 21 days, the patient exhibited complete regression of the tumors. There was no clinical evidence of recurrence without the administration of anticancer treatment. It is unique that short-term sorafenib treatment achieved a complete response.
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PMID:A complete response induced by 21-day sorafenib therapy in a patient with advanced hepatocellular carcinoma. 2385 91

Primary splenic lymphoma is rare as non-Hodgkin lymphomas. Splenic infiltration of lymphoma cells may cause splenomegaly in many cases. However, splenomegaly is caused not only by tumor involvement but also by non-tumorous disorders. One of the most prevalent non-neoplastic causes is portal hypertension mostly due to liver cirrhosis. On the other hand, liver cirrhosis may underlie various extrahepatic manifestations including development of B-cell non-Hodgkin lymphomas. Here, we report a case of primary follicular lymphoma of the spleen in a patient with liver cirrhosis related to hepatitis C and alcohol. The lymphoma was incidentally found in an enlarged spleen resected palliatively to alleviate symptomatic pancytopenia of the patient. The main characteristic of our case is an incidental finding of a rare situation brought by careful pathological examination. Our case illustrates the importance to recognize a possibility of co-occurrence of chronic liver disease and extrahepatic lymphoma.
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PMID:Primary follicular lymphoma of the spleen incidentally found in a patient with alcohol- and hepatitis C-related liver cirrhosis. 2512 Aug 38

Graft versus host disease (GVHD) is an uncommon complication following liver transplantation. In the present case report, a 53-year-old male hepatitis B virus carrier was diagnosed with primary liver cancer with post-hepatitis cirrhosis. Preoperative cytomegalovirus (CMV), Epstein-Barr virus, coxsackievirus, herpes simplex virus and autoimmune antibody series were negative. Preoperative human leukocyte antigen type was also negative. Following classic orthotropic liver transplantation, postoperative treatment included immunosuppression therapy, infection protection, anti-human immunodeficiency virus therapy and CMV infection protection therapy. Chemotherapy was initiated at day 16 following surgery. At day 26 following the transplantation, the patient developed a fever of unknown cause, and a scattered red rash was observed behind the left ear and on the neck. The patient presented with a fever of unknown cause, rash, symptoms of the digestive tract, leukocytopenia and pancytopenia. A diagnosis of GVHD was confirmed following a skin biopsy. Symptomatic therapies, including antivirals, anti-anaphylaxis drugs and steroids were administered. However, the patient succumbed to infection, acute respiratory distress syndrome and multiple organ failure at day 46 following surgery. Therefore, an effective therapeutic strategy for the treatment of GVHD following liver transplantation is yet to be established, and further research is required prior to such a regimen being developed.
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PMID:Graft versus host disease following liver transplantation: A case report. 2518 16


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