• Title/Summary/Keyword: organomegaly

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Unusual Manifestation of Immunoglobulin G4-Related Disease Involving the Upper Arm: A Case Report (상완에 발생한 면역글로불린 G4 연관 질환의 비전형적 발현: 증례 보고)

  • Jin Hee Park;Sun Joo Lee;Hye Jung Choo
    • Journal of the Korean Society of Radiology
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    • v.84 no.3
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    • pp.719-725
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    • 2023
  • Immunoglobulin G4 (IgG4)-related disease is a rare systemic fibroinflammatory condition characterized by organomegaly or tumefactive lesions associated with lymphoplasmacytic infiltration rich in IgG4 plasma cells. We report a case of IgG4-related disease involving the subcutaneous layer of the left upper arm in a 48-year-old female presenting with an unusual soft tissue mass. US and MRI showed an irregular infiltrative soft tissue mass, indicating possible malignancy or inflammation. We discuss the diagnostic criteria, histopathologic features, radiological features, and treatment of IgG4-related disease.

Prognostically Significant Fusion Oncogenes in Pakistani Patients with Adult Acute Lymphoblastic Leukemia and their Association with Disease Biology and Outcome

  • Sabir, Noreen;Iqbal, Zafar;Aleem, Aamer;Awan, Tashfeen;Naeem, Tahir;Asad, Sultan;Tahir, Ammara H;Absar, Muhammad;Hasanato, Rana MW;Basit, Sulman;Chishti, Muhammad Azhar;Ul-Haque, Muhammad Faiyaz;Khalid, Ahmad Muktar;Sabar, Muhammad Farooq;Rasool, Mahmood;Karim, Sajjad;Khan, Mahwish;Samreen, Baila;Akram, Afia M;Siddiqi, Muhammad Hassan;Shahzadi, Saba;Shahbaz, Sana;Ali, Agha Shabbir
    • Asian Pacific Journal of Cancer Prevention
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    • v.13 no.7
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    • pp.3349-3355
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    • 2012
  • Background and objectives: Chromosomal abnormalities play an important role in genesis of acute lymphoblastic leukemia (ALL) and have prognostic implications. Five major risk stratifying fusion genes in ALL are BCR-ABL, MLL-AF4, ETV6-RUNX11, E2A-PBX1 and SIL-TAL1. This work aimed to detect common chromosomal translocations and associated fusion oncogenes in adult ALL patients and study their relationship with clinical features and treatment outcome. Methods: We studied fusion oncogenes in 104 adult ALL patients using RT-PCR and interphase-FISH at diagnosis and their association with clinical characteristics and treatment outcome. Results: Five most common fusion genes i.e. BCR-ABL (t 9; 22), TCF3-PBX1 (t 1; 19), ETV6-RUNX1 (t 12; 21), MLL-AF4 (t 4; 11) and SIL-TAL1 (Del 1p32) were found in 82/104 (79%) patients. TCF3-PBX1 fusion gene was associated with lymphadenopathy, SIL-TAL1 positive patients had frequent organomegaly and usually presented with a platelets count of less than $50{\times}10^9/l$. Survival of patients with fusion gene ETV6-RUNX1 was better when compared to patients harboring other genes. MLL-AF4 and BCR-ABL positivity characterized a subset of adult ALL patients with aggressive clinical behaviour and a poor outcome. Conclusions: This is the first study from Pakistan which investigated the frequency of5 fusion oncogenes in adult ALL patients, and their association with clinical features, treatment response and outcome. Frequencies of some of the oncogenes were different from those reported elsewhere and they appear to be associated with distinct clinical characteristics and treatment outcome. This information will help in the prognostic stratification and risk adapted management of adult ALL patients.