To report a case of T-cell prolymphocytic leukemia with panuveitis as the primary presenting feature. Case report. A 46-year-old woman presented with pain and blurred vision in the right eye. [ncbi.nlm.nih.gov]

The immunophenotype CD4 /CD8- is present in 60% of cases, the CD4 /CD8 immunophenotype is present in 25%, and the CD4-/CD8 immunophenotype is present in 15% of cases. Clonal TCR gene rearrangements for the γ and δ chains are typically found. [en.wikipedia.org]

• Rigor

  Rigors responded to treatment with Demerol. Urticaria was seen in 9 patients (23%) but was usually mild and transient. No patient experienced significant fluid retention. [doi.org]

• Inguinal Mass

  Nine months after his diagnosis of T-PLL, he underwent surgical excision of a new 7 cm left inguinal mass, and was diagnosed with CD30 positive Epstein-Barr Virus (EBV)-associated DLBCL. [ncbi.nlm.nih.gov]

• Soft Tissue Mass

  Radiologic and physical examination demonstrated an ovoid soft tissue mass of the lateral external auditory canal that, after biopsy and flow cytometry studies, was found to be T-cell prolymphocytic leukemia. [ncbi.nlm.nih.gov]

• Pharyngitis

  He had a history of pharyngeal diffuse large B-cell lymphoma successfully treated with polychemotherapy including cyclophosphamide and epirubicin, 6 years before the current illness. [ncbi.nlm.nih.gov]

• Left Upper Quadrant Pain

  To present a case report of an 83-year-old female with underlying dementia and a 1-2 month history of left upper quadrant pain. [ncbi.nlm.nih.gov]

• Chemosis

  A 57-year-old man presented with bilateral conjunctival hyperemia and chemosis associated with pain. He also complained of having fatigue, general weakness, and disequilibrium. His visual acuity was 20/20 bilaterally. [ncbi.nlm.nih.gov]

  Different combinations of topical lubricants and anti-inflammatory topical eyedrops were prescribed to relieve conjunctival congestion and chemosis, without success. [meajo.org]

• Conjunctival Hyperemia

  A 57-year-old man presented with bilateral conjunctival hyperemia and chemosis associated with pain. He also complained of having fatigue, general weakness, and disequilibrium. His visual acuity was 20/20 bilaterally. [ncbi.nlm.nih.gov]

• Papular Skin Rash

  He also had a maculo- papular skin rash. There was marked leucocytosis, anemia and thrombocytopenia (WBC 445 x 103 sub/ml, Hb 8.5 gm/dl, Platelet 25 x 103 sub/microl) with 60% prolymphocytes in the peripheral blood. [ncbi.nlm.nih.gov]

  He also had a maculo- papular skin rash. There was marked leucocytosis, anemia and thrombocytopenia (WBC 445 x10 3 sub/ml, Hb 8.5gm/dl, Platelet 25x10 3 /µl) with 60% prolymphocytes in the peripheral blood. [indianjcancer.com]

  He had leg edema and papular skin rash around the neck. His chest was clear with reduced air entry over the right lower zone. Cardiovascular examinations revealed normal heart sounds. [meajo.org]

• Subcutaneous Mass

  T-PLL surrounded dermal blood vessels and appendages (n 6), diffusely replaced dermis (n 3), or formed a subcutaneous mass (n 1). Other extramedullary sites included liver and lymph nodes (3 each) and spleen, lung, and cecum (1 each). [ncbi.nlm.nih.gov]

• Intracranial Hemorrhage

  Patient 39 died suddenly, probably from intracranial hemorrhage, 2 weeks after commencing treatment. The overall response rate was 76%, with 60% CR and 16% PR (Table 2 ). [doi.org]

• Dysphasia

  An 89-year-old woman who had been treated for Hashimoto's thyroiditis for 20 years presented with a progressively enlarging thyroid mass accompanied by dyspnea and dysphasia. [ncbi.nlm.nih.gov]

• Cavitary Lesion

  He was admitted to our hospital for chemotherapy and evaluation of a pulmonary cavitary lesion by wedge resection. During sedation, the patient experienced acute respiratory failure and hypovolemic shock, from which he could not be resuscitated. [ncbi.nlm.nih.gov]

• Gastric Lesion

  Endoscopic examination of the upper gastrointestinal tract revealed hemorrhage from a gastric lesion with histological involvement. [ncbi.nlm.nih.gov]

Only 3 of 9 patients (33%) responded to treatment. Furthermore, 2 of 9 patients (22%) died while on treatment. Recruitment was terminated because of these poor results. [ncbi.nlm.nih.gov]

Patients with very bulky lymph node masses were also more resistant to treatment. [doi.org]

Conjunctival involvement with leukemia was consistent with good visual acuity; however, it portended a poor prognosis. [ncbi.nlm.nih.gov]

[…] to Dr Abhirami Vivekanandarajah, avivek27{at}gmail.com Summary T-cell prolymphocytic leukaemia (T-PLL) is an extremely uncommon haematological malignancy that has an aggressive course and a grave prognosis. [casereports.bmj.com]

Abstract T-cell prolymphocytic leukemia (T-PLL) is a rare type of post-thymic T-cell neoplasm, the etiology of which is unknown. [ncbi.nlm.nih.gov]

[…] been used for small cell variant of T-PLL Definitions Aggressive leukemia characterized by High number of small to medium-sized prolymphocytes Involvement of peripheral blood (PB), bone marrow (BM), spleen, and liver other sites Mature T-cell lineage ETIOLOGY [basicmedicalkey.com]

Constant antigenic stimulus such as a cytomegalovirus reactivation may be the underlying etiology of clonal T-LGL expansion and may contribute to cytopenias and fatigue seen in transplant patients [ 38 ]. 5. [intechopen.com]

Prolymphocytic leukemia of T-cell type (effective October 01, 2015) Signs and Symptoms Anemia Hepatosplenomegaly Thrombocytopenia Diagnostic Exams Flow cytometry Immunohistochemistry Immunophenotyping Peripheral blood smear Progression and Transformation None Epidemiology [seer.cancer.gov]

Terminology Historically, small cell variants were termed T cell chronic lymphocytic leukemia Epidemiology Rare, 2% of mature lymphocytic leukemias in adults 30 years of age Median age 65 years; range: 30 - 94 years Sites Peripheral blood, bone marrow [pathologyoutlines.com]

Synonyms Epidemiology Accounts for 2% of all small lymphocytic leukaemias of adults over 30 years. [e-immunohistochemistry.info]

[…] engagement Tcl-1 dysregulation may drive early clonal expansion or promote growth advantage Possibly most important in early stages of pathogenesis of T-PLL With clonal evolution, other molecular abnormalities may drive proliferation CLINICAL ISSUES Epidemiology [basicmedicalkey.com]

Pathophysiology T-PLL is a malignancy of post thymic T-cells with the cytogenetic abnormalities listed above. [cancertherapyadvisor.com]

Clinical Information Discusses physiology, pathophysiology, and general clinical aspects, as they relate to a laboratory test T-cell neoplasms are relatively uncommon, accounting for approximately 12% of all non-Hodgkin lymphomas. [mayomedicallaboratories.com]

Prevention Without any known preventable risk factors, there is no known preventative course to take against T-cell prolymphocytic leukemia. Resources U.S. [knowcancer.com]

BACKGROUND: Scarce systematic trial data have prevented uniform therapeutic guidelines for T-cell prolymphocytic leukemia (T-PLL). [ncbi.nlm.nih.gov]

Other therapies: Prophylactic measures to prevent tumor lysis syndrome Prophylactic antimicrobials when using alemtuzumab Monitoring for Cytomegalovirus ( CMV) reactivation with use of alemtuzumab What should you tell the patient and the family about [cancertherapyadvisor.com]

Leukemia Risk & Prevention We offer the only program in the area to offer personalized risk assessment for patients and families who may be at an increased risk for developing leukemia due to genetic factors. [uchospitals.edu]

Google Scholar Friedenson B: The BRCA1/2 pathway prevents hematologic cancers in addition to breast and ovarian cancers. BioMed Central Cancer. 2007, 7: 152-163. [casesjournal.com]