While the clinical presentation of HSTCL is variable, most patients initially present with prominent hepatosplenomegaly and thrombocytopenia in the absence of prominent lymphadenopathy ( 7 ). [jgo.amegroups.com]

In conclusion, although HSTCL patients have a poor prognosis overall, the data presented support the novel suggestions that HSTCL patients can be stratified into 2 prognostic groups, with an elevated serum bilirubin level, αβ TCR expression, and trisomy [mdanderson.elsevierpure.com]

We describe a case of an adolescent boy with hepatosplenic gamma delta T-cell lymphoma with leukemic presentation, who was diagnosed to have visceral leishmaniasis, 9 months prior to presentation at our center. [jmedicalcasereports.biomedcentral.com]

Patients presented with fatigue, hepatosplenomegaly, and pancytopenia. [pediatrics.aappublications.org]

• Splenomegaly

  Most patients had massive splenomegaly, and bone marrow showed sinusoidal involvement by lymphoma. The HSTCL cells expressed γδ T-cell receptor (TCR) in 20 (74%), αβ TCR in 5 (19%), and neither in 2 (7%) patients (1 case not assessed). [mdanderson.elsevierpure.com]

  Case report 2 A 9-year-old boy had a progressive splenomegaly, mild hepatomegaly and thrombocytopenia. The bone marrow biopsy showed hypercellular marrow without atypical lymphoid cells. Splenectomy was performed due to progressive splenomegaly. [adc.bmj.com]

  He underwent surgical resection for splenomegaly. The spleen weighed 983 g and measured 21 × 14.5 × 7 cm, with no identifiable gross lesions. The cut surface was homogeneous and red-purple (panel A). [bloodjournal.org]

  Hepatosplenic T-cell lymphoma (effective October 01, 2015) Signs and Symptoms Anemia Drenching night sweats Fatigue Fever (for no known reason) Hepatomegaly Leukopenia Pain in the chest, abdomen, or bone s (for no known reason) Skin rash or itchy skin Splenomegaly [seer.cancer.gov]

• Massive Splenomegaly

  Most patients had massive splenomegaly, and bone marrow showed sinusoidal involvement by lymphoma. The HSTCL cells expressed γδ T-cell receptor (TCR) in 20 (74%), αβ TCR in 5 (19%), and neither in 2 (7%) patients (1 case not assessed). [mdanderson.elsevierpure.com]

  A CT scan revealed a liver with cirrhotic changes without focal lesions and massive splenomegaly with axillary, retroperitoneal and inguinal lymphadenopathy ( Figure 1 ). Bone marrow and lymph node biopsies were performed. [jgo.amegroups.com]

  An acute form of T γ lymphoproliferative disease presenting with massive splenomegaly – importance of immunophenotyping for diagnosis Clin Immunol Immunopathol 1993 67 : 100–108 35. Lei KI-K, Chow JH-S, Johnson PJ. [nature.com]

• Fever

  (Pentostatin, ICE) + auto-SCT 7 Humphreys et al [ 19 ] 2008 27, F 5 (AZT) Fever, nights sweats, pancytopenia, HSM Interferon-α > 31 Zeidan et al [ 20 ] 2007 31, M 6 (6MP) 1 Chills, SM, fever, pancytopenia Ch. [wjgnet.com]

  His clinical course involved multiple hospitalizations complicated by hyponatremia, neutropenic fevers and pulmonary emboli. [jgo.amegroups.com]

  Prophylaxis with antibiotics may be appropriate in some, and patients should always contact their oncologist if they develop a fever. [knowcancer.com]

• Weight Loss

  loss (for no known reason) Diagnostic Exams Flow cytometry Immunohistochemistry Immunophenotyping Splenectomy Progression and Transformation Relapses seen in vast majority of patients who respond initially to chemotherapy Epidemiology and Mortality Age [seer.cancer.gov]

  Patients with HSTCL frequently have generalized symptoms, such as fatigue, significant night sweats, weight loss and unexplained fevers. [tcllfoundation.org]

• Pathologist

  Edited by four leading surgical pathologists - Noel Weidner, MD, Richard J. Cote, MD, Saul Suster, MD and Lawrence M. [books.google.com]

  However, in some cases, making the diagnosis is challenging and requires review by specialized pathologists. [tcllfoundation.org]

  Uses expanded tables to outline specific differential diagnostic points helpful for surgical pathologists. Discusses the key molecular aspects of tumor progression and risk assessment in all chapters that cover neoplastic disorders. [books.google.it]

• Chills

  (Pentostatin, ICE) + auto-SCT 7 Humphreys et al [ 19 ] 2008 27, F 5 (AZT) Fever, nights sweats, pancytopenia, HSM Interferon-α > 31 Zeidan et al [ 20 ] 2007 31, M 6 (6MP) 1 Chills, SM, fever, pancytopenia Ch. [wjgnet.com]

  Patients with Sézary syndrome may also exhibit general, nonspecific symptoms including fevers, unintended weight loss, chills, fatigue, and malaise. [rarediseases.org]

• Nausea

  Supportive treatment: Steroids, blood transfusions, anti-nausea medications, and antibiotics, may also be used. In combination with other treatment measures, these can help combat the symptoms of immune deficiency. [dovemed.com]

  This called for a celebration even the oncologist announced this as Very Good news to us, so now she is on MTX ( methetraxte) once a week 12,5mg, but the three days following the dose she is wipped, she has unbearable nausea, cannot eat, and sleeps all [cancerresearchuk.org]

  Acupuncture has been helpful in relieving musculoskeletal symptoms, as well as in controlling nausea and vomiting associated with chemotherapy. [emedicinehealth.com]

  The most frequent symptom at presentation is abdominal pain or discomfort, occurring in 39–70% of patients ( 3 ), and other symptoms include fever, loss of weight and night sweats (also known as ‘B’ symptoms), nausea, vomiting, asthenia or itching. [spandidos-publications.com]

• Vomiting

  The patient had no history of fever, vomiting, night sweats, chest pain, icterus, diarrhea or stool blood loss. [spandidos-publications.com]

  It is most commonly used in combination with chemotherapy The radiation may be administered by a machine placed outside the body, or by putting a radioactive material inside the body The side effects of radiation therapy include nausea, vomiting, fatigue [dovemed.com]

  Acupuncture has been helpful in relieving musculoskeletal symptoms, as well as in controlling nausea and vomiting associated with chemotherapy. [emedicinehealth.com]

• Loss of Appetite

  […] of appetite and weight loss Fatigue High temperatures and excessive night sweats ‘Non-painful’ swelling of lymph nodes in the neck, armpits, or groin, may be present; though, this is an uncommon finding Abnormal liver function tests, irregular lymphocytes [dovemed.com]

  Other side effects may include nausea and vomiting, loss of appetite, hair loss, sores in the mouth and digestive tract, fatigue, muscle aches, and changes in fingernails and toenails. [emedicinehealth.com]

  Review of systems was positive for jaundice, mild abdominal pain, diarrhea, loss of appetite and subjective weight loss since one month. [ispub.com]

• Left Upper Quadrant Pain

  History of present illness was notable for a 6-month history of nausea, emesis, early satiety, and severe left upper quadrant abdominal pain that radiated to her shoulder. [hematologyandoncology.net]

• Right Upper Quadrant Pain

  PHL typically occurs in middle aged men, and usually the chief presenting symptoms are non specific which includes right upper quadrant pain, B symptoms like fever and weight loss and constitutional symptoms. [tropicalgastro.com]

• Thrombosis

  Complications Hepatomegaly Hepatic failure Portal vein thrombosis Splenomegaly Splenic infarction Spleen rupture Splenic vein thrombosis Anemia Thrombocytopenia Neutropenia Neurological dysfunction if metastasizes to brain. [wikidoc.org]

  Acute diagnostics of ascites, heart, lung or abdomen did not reveal any septic focus, thrombosis, pericardial effusion or intestinal perforation. The patient died 5 hours after intubation from acute cardiovascular failure. [ghrnet.org]

  […] hepatitis C, fatty liver, long standing alcohol abuse); blood cancers (lymphoma, leukemia, myelofibrosis); infections (mononucleosis, bacterial endocarditis, malaria, AIDS, mycobacterium, leishmania); abnormal blood flow and congestion (splenic vein thrombosis [emedicinehealth.com]

• Hypotension

  This was complicated by hypotension and multiorgan failure, including renal impairment, abnormal liver function and myocardial injury, and he required intensive care. [mja.com.au]

• Hepatomegaly

  It occurs mainly in young adults presenting with splenomegaly and most often with hepatomegaly but without lymphadenopathy. The lymphoma cells are usually CD3 +, CD2 +, CD5 −, CD7 +/−, CD4 −, CD8 +/−, and CD56 +/−. [bloodjournal.org]

  ICD-10 Codes C84.5 Other and unspecified T-cell lymphomas Corresponding ICD-10-CM Codes (U.S. only) C86.1 Hepatosplenic T-cell lymphoma (effective October 01, 2015) Signs and Symptoms Anemia Drenching night sweats Fatigue Fever (for no known reason) Hepatomegaly [seer.cancer.gov]

  Case report 2 A 9-year-old boy had a progressive splenomegaly, mild hepatomegaly and thrombocytopenia. The bone marrow biopsy showed hypercellular marrow without atypical lymphoid cells. Splenectomy was performed due to progressive splenomegaly. [adc.bmj.com]

  6 (40) Biopsy-proven liver involvement in patients without hepatomegaly 3 (20) Abnormal LFTs 6 (40) Adenopathy 2 (13) Peripheral blood involvement 4 (27) B symptoms Fever 10 (67) Night sweats 9 (60) Weight loss 8 (53) Cytopenias Anemia 8 (73) Thrombocytopenia [academic.oup.com]

• Jaundice

  Review of systems was positive for jaundice, mild abdominal pain, diarrhea, loss of appetite and subjective weight loss since one month. [ispub.com]

  Symptoms include fever, fatigue, jaundice, and infections. Tests often reveal abnormal liver function and reduced peripheral blood cells. [rosenfeldinjurylawyers.com]

  She consulted her general practitioner and was admitted to a primary care hospital for acute hepatic failure with jaundice (total bilirubin 6.4 mg/dL, direct bilirubin 5.3 mg/dL), coagulopathy (INR>6.3) and encephalopathy grade 2-3. [ghrnet.org]

  Systemic symptoms of hepatosplenic T-cell lymphoma include fatigue, fever, recurrent infections, and jaundice due to liver involvement. Abnormal liver function tests are also common. [knowcancer.com]

• Asthenia

  The most frequent symptom at presentation is abdominal pain or discomfort, occurring in 39–70% of patients ( 3 ), and other symptoms include fever, loss of weight and night sweats (also known as ‘B’ symptoms), nausea, vomiting, asthenia or itching. [spandidos-publications.com]

Provides all the necessary tools to make a comprehensive diagnostic workup including data from ancillary techniques and molecular findings whenever appropriate. [books.google.it]

Therapeutic pleural tapping of approximately one-liter fluid revealed an exudative fluid with negative workup for TB and negative cytology. [ijpmonline.org]

Serological workup for Echinococcus, Histoplasma, E. histolytica, Cryptococcus, Leishmania, Leptospira, HTLV I/II and syphilis was negative. There was positive IgG antibody against EBV and CMV. [ispub.com]

Initial laboratory workup showed abnormal liver-function tests and elevated total bilirubin; other results are shown in Table 1. Serological tests were negative for hepatitis viruses A, C, D and E. [jgo.amegroups.com]

• Enlargement of the Liver

  When doctors start evaluating patients with HSTCL, they frequently find very low blood cell counts and abnormalities in liver function on blood tests; enlarged spleen and liver on the CT-scans (of note: lymph nodes or other organs are very rarely enlarged [tcllfoundation.org]

  In addition, there are many, more common causes of an enlarged liver and/or spleen and abnormal liver function tests; therefore, this diagnosis is often not suspected. [knowcancer.com]

  […] and spleen enlargement Loss of appetite and weight loss Fatigue High temperatures and excessive night sweats ‘Non-painful’ swelling of lymph nodes in the neck, armpits, or groin, may be present; though, this is an uncommon finding Abnormal liver function [dovemed.com]

This follow-up period is to: see how you are feeling check you have recovered from your treatment check there are no signs of the lymphoma relapsing look for any late effects (side effects that develop months or years after treatment) of your treatment [essais-cliniques-lymphomes.fr]

Back to top Treatment T-cell lymphomas are rare, so it is difficult to determine which treatment gives the best outcome. You might be asked if you would like to take part in a clinical trial to help test new and current treatments. [lymphomas.org.uk]

[…] patients, AIH will require lifelong treatment. 12 Alternative medical treatment is available due to promising results of, for example, mycophenolatemofetil, which was shown to be an effective first-line therapy in an uncontrolled trial. 10 However, mycophenolatemofetil [pediatrics.aappublications.org]

However, there are treatment options, but the aggressive cancer requires aggressive treatment. [rosenfeldinjurylawyers.com]

Patients have a poor prognosis, and there is no standard of care. We evaluated 28 HSTCL patients to determine factors that may be associated with outcome. There were 19 men and 9 women with a median age of 32.5 years. [mdanderson.elsevierpure.com]

Blum Springer, 6 Feb 2015 - Medical - 344 pages This book provides clinical practitioners and the research community with detailed information on the diagnosis, prognosis, and treatment of non-Hodgkin lymphoma, taking into account the significant growth [books.google.com]

Conclusion: It is an infrequent condition with an unfavorable prognosis. [scienceopen.com]

Prognosis There may be initial response to chemotherapy, but relapse is the norm with a median survival of less than 2 years. [e-immunohistochemistry.info]

He was subsequently diagnosed with cirrhosis of unknown etiology. With evidence of active Babesial infection, he was treated with atovaquone and discharged home. [jgo.amegroups.com]

Laboratory findings for infectious etiology were nondiagnostic. He underwent surgical resection for splenomegaly. The spleen weighed 983 g and measured 21 × 14.5 × 7 cm, with no identifiable gross lesions. [bloodjournal.org]

Since the patient had fever and diarrhea at presentation in the setting of extensive travel history, infectious etiology was high in the list of differential diagnosis initially. [ispub.com]

The etiology is unknown but potential lymphomatogenic risk factors include 4 : viral infection, e.g. EBV, HTLV-1, HIV, HCV, HSV bacterial infection, e.g. [radiopaedia.org]

The work is subdivided into epidemiology, pathogenesis, pathology, imaging, and therapy of the non-Hodgkin lymphomas. [books.google.com]

Synonyms Epidemiology Rare, representing Clinical features There is marked hepatosplenomegaly without lymphadenopathy. Bone marrow involvement results in anaemia and thrombocytopenia. Histopathology The cells are medium size and monotonous. [e-immunohistochemistry.info]

Splenomegaly Thrombocytopenia Weight loss (for no known reason) Diagnostic Exams Flow cytometry Immunohistochemistry Immunophenotyping Splenectomy Progression and Transformation Relapses seen in vast majority of patients who respond initially to chemotherapy Epidemiology [seer.cancer.gov]

Epidemiology Rare, < 1% of all non Hodgkin lymphomas Adolescents and young adults, median age ~35 years Male predominance Sites Spleen, liver, bone marrow Usually no significant nodal involvement Peripheral blood involvement late in disease; may have [pathologyoutlines.com]

A better understanding of the pathophysiology of HSTCL and new therapeutic strategies are needed. [scienceopen.com]

Pathophysiology Hepatosplenic T-cell lymphoma is a peripheral T-cell lymphoma, a type of non Hodgkin's lymphoma [1]. It is also known as ‘gamma-delta hepatosplenic T-cell lymphoma [2]. [wikidoc.org]

"Alice in Wonderland Syndrome: A Clinical and Pathophysiological Review". BioMed Research International. 2016: 1–10. doi:10.1155/2016/8243145. PMC 5223006. PMID 28116304. ^ Nussinovitch M, Prais D, Volovitz B, Shapiro R, Amir J (September 2003). [en.wikipedia.org]

Primary Prevention There are no established measures for the primary prevention of hepatosplenic t cell lymphoma. Secondary Prevention There are no established measures for the secondary prevention of hepatosplenic t cell lymphoma. [wikidoc.org]

It is thought that the immune suppressing drugs needed to prevent rejection of the transplanted organ enhance the risk of this subtype of NHL. Immune suppression is a risk factor for other subtypes of NHL as well. [knowcancer.com]

IMURAN ® (azathioprine) is a drug used to treat adult rheumatoid arthritis and help prevent kidney transplant rejection. PURINETHOL ® (mercaptopurine) is a drug approved to treat cancer (leukemias). [healthycanadians.gc.ca]