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Hepatosplenic Lymphoma


Presentation

  • 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]
  • 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]
  • 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]
  • , secondary rather than Primary MF is more likely to be present in our case.[avicennajmed.com]
Splenomegaly
  • 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]
  • 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]
  • 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]
  • On ultrasound of abdomen: Hepatomegaly Splenomegaly CT scan Tumor mass can be seen in liver or spleen or both. MRI Tumor mass can be seen in liver or spleen or both.[wikidoc.org]
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]
  • Blood 2014 124:677; doi: A 30-year-old man with an unremarkable medical history presented with fever, anemia, and thrombocytopenia, along with hepatosplenomegaly, for 1 month. Laboratory findings for infectious etiology were nondiagnostic.[bloodjournal.org]
  • His clinical course involved multiple hospitalizations complicated by hyponatremia, neutropenic fevers and pulmonary emboli.[jgo.amegroups.com]
  • VL is a systemic disease with gradual onset of fever, pancytopenia, hepatosplenomegaly, and weight loss. The incubation period can range from weeks to a year. VL is fatal if left untreated [ 6 ].[jmedicalcasereports.biomedcentral.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]
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]
Congestive Heart Failure
  • Our patient died due to acute congestive heart failure, caused by diffuse neoplastic myocardial infiltration with a proliferation fraction (MIB1) of up to 40% (Figure 3).[ghrnet.org]
  • heart failure); Gaucher disease (a lipid storage disease); blood cell disorders (sickle cell anemia, thalassemia, spherocytosis); inflammatory disease (lupus, rheumatoid arthritis); idiopathic thrombocytopenic purpura (ITP); and polycythemia vera.[emedicinehealth.com]
High Fever
  • Case report 1 A 5-month-old boy was referred with a history of remittent high fever and progressive hepatosplenomegaly. He was a term twin born from an in vitro fertilized pregnancy.[adc.bmj.com]
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]
  • 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]
  • Acupuncture has been helpful in relieving musculoskeletal symptoms, as well as in controlling nausea and vomiting associated with chemotherapy.[emedicinehealth.com]
Vomiting
  • 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]
  • The patient had no history of fever, vomiting, night sweats, chest pain, icterus, diarrhea or stool blood loss.[spandidos-publications.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]
  • Review of systems was positive for jaundice, mild abdominal pain, diarrhea, loss of appetite and subjective weight loss since one month.[ispub.com]
  • Depending on how and where the radiation is administered, it may cause certain side effects such as fatigue , loss of appetite, nausea , diarrhea , and skin problems.[emedicinehealth.com]
Left Upper Quadrant Pain
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]
  • On ultrasound of abdomen: Hepatomegaly Splenomegaly CT scan Tumor mass can be seen in liver or spleen or both. MRI Tumor mass can be seen in liver or spleen or both.[wikidoc.org]
Insect Bite
  • Alcohol intake, drug abuse, sexual risk behavior, insect bites, and close contact to animals were denied. Except for low doses of ibuprofen and acetylsalicylic acid she denied any regular medication or allergies. Medical history was otherwise empty.[ghrnet.org]
Tinnitus
  • He developed bilateral tinnitus since day 10 with evidence of high-frequency sensorineural hearing loss on audiometry examination.[ijpmonline.org]
Insomnia
  • […] people, 20.00% Irritable Bowel Syndrome: 11 people, 13.75% Chemotherapy: 5 people, 6.25% Colitis (inflammation of colon): 4 people, 5.00% Pain: 4 people, 5.00% Stress And Anxiety: 3 people, 3.75% Diabetes: 3 people, 3.75% Eye Disorder: 3 people, 3.75% Insomnia[ehealthme.com]
Irritability
  • : 9.09 % Top conditions involved for these people *: Ulcerative Colitis (inflammatory bowel disease (ibd). it causes swelling, ulcerations, and loss of function of the large intestine): 32 people, 40.00% Inflammatory Bowel Disease: 16 people, 20.00% Irritable[ehealthme.com]
Encephalopathy
  • 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]

Workup

  • Therapeutic pleural tapping of approximately one-liter fluid revealed an exudative fluid with negative workup for TB and negative cytology.[ijpmonline.org]
  • 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]
  • Provides all the necessary tools to make a comprehensive diagnostic workup including data from ancillary techniques and molecular findings whenever appropriate.[books.google.it]
  • 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]
Mediastinal Shift
  • (f) These CD2, CD7, CD56 positive events are negative for alpha-beta/gamma-delta T-cell receptors Click here to view X-ray chest-posteroanterior showed massive left pleural effusion with mediastinal shift to the right.[ijpmonline.org]
Pericardial Effusion
  • 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]
Bicytopenia
  • It can be easily misdiagnosed as acute leukemia on morphology as well as flow-cytometry, though massive hepatosplenomegaly with bicytopenia and no lymphadenopathy is highly suspicious of a diagnosis of HSTCL, especially in young individuals.[ijpmonline.org]
Normocytic Anemia
  • Peripheral blood count showed normocytic anemia and thrombocytopenia with absolute lymphopenia (0.1/nL, normal range 1.0-4.8), suggestive for drug-induced bone marrow toxicity.[ghrnet.org]

Treatment

  • Nevertheless, to date no such combination treatments have been employed yet in this entity [ 1 , 2 , 5 ].[annonc.oxfordjournals.org]
  • […] 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]
  • 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.[lymphoma-action.org.uk]

Prognosis

  • 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]
  • 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]
  • HSTL exhibits a marked chemoresistance to currently used regimens, a rapidly progressive behavior, and dismal prognosis. Patients with post-transplant HSTL exhibit an especially poor outcome. Standard treatment has yet to be established.[ncbi.nlm.nih.gov]

Etiology

  • 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]

Epidemiology

  • 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 blastic[pathologyoutlines.com]
Sex distribution
Age distribution

Pathophysiology

  • 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]
  • Conclusion: A better understanding of the pathophysiology of HSTCL and new therapeutic strategies are needed. introduction Hepatosplenic T-cell lymphoma (HSTCL) is a rare peripheral T-cell lymphoma.[academic.oup.com]
  • For discussion of individual subtypes of NHL, see the following: Pathophysiology NHLs are tumors originating from lymphoid tissues, mainly of lymph nodes.[emedicine.medscape.com]

Prevention

  • 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]
  • 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]
  • 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]

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