(February 27, 2018) An Unusual Presentation of Primary Hepatic Diffuse Large B-cell Lymphoma of the Liver. [cureus.com]

However, in our case, hepatomegaly was present. [onlinelibrary.wiley.com]

Portal hypertension was, however, not present. Exploratory laparotomy revealed nodular regenerative hyperplasia of the liver. [keio.pure.elsevier.com]

Satellite nodules were seen at presentation in one case and developed after presentation in two others. Other features, such as enhancement after contrast administration, necrosis, and calcification, were variable. [ajronline.org]

Sub-acute liver failure is an uncommon presentation of primary hepatic lymphoma. Herein, we present a patient who presented with sub-acute liver failure and jaundice due to primary hepatic follicular lymphoma. [journals.sagepub.com]

• Hodgkin Lymphoma

  Ct of nodular hyperplasia of the liver in non-hodgkin lymphoma. Journal of Computer Assisted Tomography, 15 (6), 1031-1034. [keio.pure.elsevier.com]

  […] and non-Hodgkin lymphomas, Leuk. [scipress.com]

  22 [Primary non-Hodgkin lymphoma of the liver in HIV infected patient: case-report]. 38 Walter T...Trepo C 15276292 2004 23 [Primary liver lymphoma. [malacards.org]

  Primary non-Hodgkin’s lymphoma of the liver. Crit Rev Oncol Hematol. 2005;53:199-207. Avlonitis VS, Linos D. Primary hepatic lymphoma: a review. Eur J Surg. 1999;165:725-9. [ijsurgery.com]

  B-cell non-Hodgkin lymphoma is most commonly associated with this entity, in approximately 60% of PHLs, compared to T-cell non-Hodgkin lymphoma with only 30%. 4 The pathogenesis of PHL is unknown, but chronic antigen stimulation appears to play a role [elsevier.es]

• Fever

  Clinical history is not specific made of jaundice, fever and general alteration (4). Biology shows hepatic chemical disorders. [tunisiechirurgicale.com]

  Hepatomegaly is found in most patients with constitutional symptoms (e.g., fever, night sweats and weight loss) appear in 37%, fever in 86%, weight loss in 57% and jaundice in 4% [ 4 - 7 ]. [omicsonline.org]

  Risk Factors for SOS Patient Related Transplant Related Age Allogenic transplant Malignant disease Donor type Disease relapse Bone marrow–derived stem cell origin Status of the liver (e.g., cirrhosis, fibrosis) Fever in conditioning High AST/ALT ratio [aasldpubs.onlinelibrary.wiley.com]

  PHL presents with nonspecific symptoms such as right upper quadrant or epigastric pain, fatigue, anorexia, nausea, weight loss, or fever. [onlinelibrary.wiley.com]

  Symptoms may include any of the following: Feeling very tired all the time Fever and chills that come and go Itching all over the body that cannot be explained Loss of appetite Drenching night sweats Painless swelling of the lymph nodes in the neck, armpits [medlineplus.gov]

• Anemia

  The result is anemia, or low red blood cell count. * American Cancer Society, Cancer Facts & Figures 2014 Classification of Lymphomas Lymphomas are graded as low, intermediate and high depending on the kind of lymphoma cells present and how they affect [cedars-sinai.edu]

  […] following procedures will usually be done: Blood chemistry tests including protein levels, liver function tests, kidney function tests, and uric acid level Bone marrow biopsy CT scans of the chest, abdomen, and pelvis Complete blood count (CBC) to check for anemia [medlineplus.gov]

  […] known as hepatomegaly Nausea and vomiting Abdominal pain Easy bruising or bleeding as a result of liver failure Unintentional weight loss; changes in appetite Fatigue and weakness, headache High temperatures and excessive night sweats (may be recurrent) Anemia [dovemed.com]

  Over the course of hospitalization, patient’s condition deteriorated with worsening coagulopathy, neutropenia, and anemia requiring multiple transfusions of blood products including factor concentrates. [journals.sagepub.com]

  Routine laboratory investigations were unremarkable, except for anemia (hemoglobin 10.7 g/dL). Serum alfa fetoprotein (AFP) level, serum lactate dehydrogenase (LDH) level and serum carcinoembryonic antigen (CEA) levels were within normal limits. [jcytol.org]

• Constitutional Symptom

  Hepatomegaly is found in most patients with constitutional symptoms (e.g., fever, night sweats and weight loss) appear in 37%, fever in 86%, weight loss in 57% and jaundice in 4% [ 4 - 7 ]. [omicsonline.org]

  He presented to our institution with right upper quadrant pain and abdominal swelling with constitutional symptoms and lower extremity swelling. [cureus.com]

  […] vomiting, upper abdominal pain or discomfort, [2] [3] and approximately one-third report constitutional symptoms including fever, myalgias, and weight loss. [ncbi.nlm.nih.gov]

  J Cytol 2015;32:36-8 Primary hepatic lymphoma (PHL) is a rare type of primary liver tumor and an unusual type of lymphoma. [1] It usually presents with constitutional symptoms, hepatomegaly but without lymphadenopathy and extrahepatic (i.e., bone marrow [jcytol.org]

• Falling

  He reported no history of trauma or falls. He had not been prescribed anticoagulants or antiplatelet agents. [surgicalcasereports.springeropen.com]

• Nausea

  It can cause abdominal pain, nausea and vomiting, weight loss, and other general signs and symptoms, such as fever, fatigue, and appetite loss Chemotherapy, surgery, radiation therapy, and other treatment measures may be used for treating Lymphoma of [dovemed.com]

  PHL presents with nonspecific symptoms such as right upper quadrant or epigastric pain, fatigue, anorexia, nausea, weight loss, or fever. [onlinelibrary.wiley.com]

  Many patients with cancer, especially in the advanced stages, are unable to ingest large meals and suffer with nausea and poor appetite. [liverdoctor.com]

  The liver is the major reticuloendothelial organ, and hepatic involvement secondary to systemic NHL is common, such that 40% of patients with NHL posses liver involvement. [1] The majority of the patients with PHL report vague symptoms such as nausea, [ncbi.nlm.nih.gov]

  Symptoms of stage 4 lymphoma can include: enlarged lymph nodes under the skin fatigue chills loss of appetite itching a persistent cough shortness of breath chest pain abdominal bloating early satiety easy bruising or bleeding frequent infections nausea [medicalnewstoday.com]

• Vomiting

  It can cause abdominal pain, nausea and vomiting, weight loss, and other general signs and symptoms, such as fever, fatigue, and appetite loss Chemotherapy, surgery, radiation therapy, and other treatment measures may be used for treating Lymphoma of [dovemed.com]

  We present a 37-year-old male with worsening jaundice, abdominal pain, and vomiting who was diagnosed with hepatic peripheral T-cell lymphoma with a Ki-67 of 70%. [wjgnet.com]

  In this case report, we present a 58-year-old lady with poor performance status who presented with obstructive jaundice, epigastric pain, and bilious vomiting. [ehj.eg.net]

  The liver is the major reticuloendothelial organ, and hepatic involvement secondary to systemic NHL is common, such that 40% of patients with NHL posses liver involvement. [1] The majority of the patients with PHL report vague symptoms such as nausea, vomiting [ncbi.nlm.nih.gov]

  Symptoms of stage 4 lymphoma can include: enlarged lymph nodes under the skin fatigue chills loss of appetite itching a persistent cough shortness of breath chest pain abdominal bloating early satiety easy bruising or bleeding frequent infections nausea or vomiting [medicalnewstoday.com]

• Abdominal Bloating

  Symptoms of stage 4 lymphoma can include: enlarged lymph nodes under the skin fatigue chills loss of appetite itching a persistent cough shortness of breath chest pain abdominal bloating early satiety easy bruising or bleeding frequent infections nausea [medicalnewstoday.com]

• Hypertension

  Portal hypertension was, however, not present. Exploratory laparotomy revealed nodular regenerative hyperplasia of the liver. [keio.pure.elsevier.com]

  Case Presentation A 64-year-old male with a past medical history of morbid obesity, Roux-en-Y gastric bypass, cholecystectomy, type II diabetes mellitus, and hypertension presented with lower extremity and abdominal swelling, right upper quadrant pain [cureus.com]

  The patient’s history was also significant for hypothyroidism, hypertension, gout, type 2 diabetes mellitus, hyperlipidemia, and mitral valve disease without regurgitation. [ncbi.nlm.nih.gov]

  His past medical included hypertension and diabetes well controlled with medications, but no autoimmune disease. On physical examination, the patient was jaundiced with fever (39°). Laboratory tests showed cholestasis and cytolysis. [tunisiechirurgicale.com]

  From the Department of Gastroenterology, Liver and Portal Hypertension Surgery Unit, Hospital das Clínicas, Faculty of Medicine, University of São Paulo. [scielo.br]

• Jaundice

  Rarely, jaundice with acute liver failure is the first clinical manifestation of lymphoma. [aasldpubs.onlinelibrary.wiley.com]

  Prognosis is better if diagnosed and treated early (probably before performance worsens and jaundice increases or appears). [ehj.eg.net]

  We present a 37-year-old male with worsening jaundice, abdominal pain, and vomiting who was diagnosed with hepatic peripheral T-cell lymphoma with a Ki-67 of 70%. [wjgnet.com]

  Jaundice is less frequent noticed in progressed disease, in cirrhotic patients and in case of compressive lymph nodes. [tunisiechirurgicale.com]

  Klatskin tumor or hilar cholangiocarcinoma should also be considered in a patient with this presentation as an obstruction at the junction of the left and right hepatic ducts can cause jaundice and acute liver failure [5]. [cureus.com]

Table 2 Laboratory data A workup for causes of increased LFTs had been initiated on HD 1. [dovepress.com]

Figure 1 Imaging workup. [casereports.bmj.com]

[…] lymphomas. [4] 68 cases of primary MALTomas have been reported in literature till date. [3] Mean age of presentation is the sixth decade with slight female predilection. [2] Hepatic MALTomas are mostly incidentally detected during surgery or on imaging workup [ijpmonline.org]

This case highlights the importance of pursuing a liver biopsy early in the workup for abnormal liver tests of an unclear etiology, even in the absence of a liver lesion on imaging, as in some instances a more expedited diagnosis can improve prognosis [cureus.com]

A thorough workup is helpful including HBsAg, HCV antibodies, EBV IgM or PCR, and HIV serology. [ncbi.nlm.nih.gov]

Recurring tumors have worse prognosis compared to tumors that do not recur Response to treatment: Tumors that respond to treatment have better prognosis compared to tumors that do not respond to treatment Progression of the condition makes the outcome [dovemed.com]

The laboratory, imaging, histologic immunohistochemical results, treatment schema and outcome of them were collected and summarized in this article. [tcr.amegroups.com]

Since chemotherapy is the treatment of first choice for lymphoma, adjuvant chemotherapy should be given to patients for optimal treatment[ 1 ]. The existing literature on PHL reveals the difficulties involved in diagnosis and treatment. [ncbi.nlm.nih.gov]

Treatment usually consists of high-dose radiotherapy, chemotherapy or a combination of both. Intensive treatment involves risk, but recent studies suggest that such treatment may induce high rates of remission. [cedars-sinai.edu]

The optimal treatment of PHL is not yet defined. Surgical treatment, radiotherapy, and chemotherapy were all reported as treatment modalities alone or in combination. [ehj.eg.net]

International Prognostic Index In addition to stage, a scale called the International Prognostic Index (IPI) is important in determining the prognosis of aggressive lymphomas. Prognosis is the chance of recovery. [cancer.net]

Generally, primary liver lymphoma has a poor prognosis with a median survival of 15 months ranging from 3 to 123 months (14). The factors determining the prognosis are the pathological subtype and the pattern of liver involvement (16). [tunisiechirurgicale.com]

As PHL was often misdiagnosed as other hepatic diseases and had a poor prognosis, it should be given enough attention and specific treatment strategy was recommended. [tcr.amegroups.com]

The prognosis for patients with PHL relates to the specific disease entity. [oapublishinglondon.com]

Etiology of MALToma is unclear. Chronic inflammation is known to be the possible trigger including chronic gastritis associated with H. pylori infection. [ijpmonline.org]

In the setting of worsening symptoms and abnormal liver enzymes of unknown etiology, clinicians should consider performing a differential liver biopsy. [wjgnet.com]

The etiology of primary hepatic DLBCL is unknown. [cureus.com]

Etiology Certain viruses such as hepatotropic and non-hepatotropic viruses share links with the causation of the PHL. [ncbi.nlm.nih.gov]

[…] diffuse histiocytic lymphoma (5%), lymphoma of the mucosa associated with lymphoid tissue type, anaplastic large cell lymphoma, mantle cell lymphoma and T-cell-rich B-cell lymphoma. [6] The exact cause of PHL is unknown, although an association with viral etiology [jcytol.org]

Therefore, since the Birmingham (UK) group emphasized that the establishment of a histopathology data pool improves epidemiological analysis, this paper is based on such a pool used by the Igbo ethnic group [5, 6]. [symbiosisonlinepublishing.com]

To evaluate which patients with NHL are at the highest risk of developing an infection-related second cancer, the researchers reviewed 17 Surveillance, Epidemiology, and End Results (SEER) Program registries to identify 127,044 adults who received their [ashclinicalnews.org]

National Cancer Institutes, Surveillance, Epidemiology, and End Results Program. Available online at http://seer.cancer.gov/statfacts/html/nhl.html. Accessed July 21, 2015. SEER Training Modules, WHO Classification. [labtestsonline.it]

Increasing incidence and descriptive epidemiology of extranodal non-Hodgkin lymphoma in parts of England and Wales. Hematol J. 2002;3(2):95–104. 8- Maher MM, McDermott SR, Fenlon HM, Conroy D, O’Keane JC, Carney DN, et al. [tunisiechirurgicale.com]

Surveillance Epidemiology and End Results (SEER). Bethesda, MD: National Cancer Institute. Archived from the original on 26 September 2009. Retrieved 3 November 2009. Table 1.4: Age-Adjusted SEER Incidence and U.S. [en.wikipedia.org]

Pathophysiology Despite a well established underlying pathophysiology in the pathogenesis of other forms of lymphoma; both non-Hodgkin and Hodgkin lymphoma, the pathophysiology underlying primary hepatic lymphoma is largely underdetermined. [ncbi.nlm.nih.gov]

Bilirubin ≥2 mg/dL (∼34 μmol/L) before day 21 post‐HSCT and at least two of the following: • Hepatomegaly • Ascites • Weight gain ≥5% from baseline Summary Assessing liver dysfunction in the context of lymphoma or after HSCT requires an understanding of the pathophysiology [aasldpubs.onlinelibrary.wiley.com]

“It seems intuitive to eradicate HCV to prevent HCV-NHL as very few patients who attained an SVR developed such cancer,” the researchers wrote. “Our findings suggest the need for early AVT in infected patients. [healio.com]

A high index of suspicion and liver biopsy early in the course of liver dysfunction may establish the diagnosis and allow rapid initiation of chemotherapy to prevent a fatal outcome. [ejcrim.com]

How can Lymphoma of Liver be Prevented? Currently, it is not possible to prevent Lymphoma of Liver. However, controlling certain factors may help lower one’s risk for the condition. [dovemed.com]

[…] chemotherapy or radiation therapy include: Bone marrow diseases (such as leukemia) Heart disease Inability to have children ( infertility ) Lung problems Other cancers Thyroid problems Keep following up with a provider who knows about monitoring and preventing [medlineplus.gov]

Burkitt lymphoma may spread to the brain and spinal cord and treatment to prevent its spread may be given. [cancer.gov]