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B-Cell Prolymphocytic Leukemia
B-PLL

Presentation

Nine months after the initial presentation, the patient's hemoglobin level fell to 5.7 g/dL. Coombs' test was positive and marked hypoplasia of erythroid precursors was detected in his bone marrow. [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]

Entire Body System

  • Fatigue

    An 86-year-old man underwent a medical examination for lumbago and general fatigue at another hospital in June 2007. A compressed lumbar fracture and splenomegaly were found using computed tomography (CT). [ncbi.nlm.nih.gov]

    We ask about general symptoms (anxious mood, depressed mood, fatigue, pain, and stress) regardless of condition. Last updated: May 13, 2019 [patientslikeme.com]

    Common leukemia symptoms are: persistent fatigue, weakness; frequent infections; weight loss without trying; swollen lymph nodes, enlarged liver or spleen; easy bleeding or bruising; tiny red spots in the skin (petechiae); and bone pain or tenderness. [publichealth.va.gov]

    Most patients present with symptoms pancytopenia (fatigue, infection, easy bruising) or splenomegaly, but with little lymphadenopathy. Two hairy cells positive for acid phosphatase despite attempted tartrate inhibition (TRAP positive). [web.archive.org]

Skin

  • Subcutaneous Nodule

    The patient was then treated with single-agent rituximab and returned in 16 months with relapse as a right chest wall subcutaneous nodule. A subsequent positron emission tomography scan revealed widespread bony lesions. [doi.org]

  • Subcutaneous Mass

    Recurrence as nodal disease was seen in one patient (case 2) with MYC amplification (more than five signals), and recurrence as a subcutaneous mass was seen in another patient (case 5) with MYC rearrangement. [doi.org]

Workup

Further workup revealed splenomegaly and liver cirrhosis, with no B symptoms or lymphadenopathy per imaging studies. Peripheral blood flow cytometric analysis was consistent with B-cell leukemia. [doi.org]

Treatment

Talk with your doctor about the goals of each treatment and what you can expect while receiving the treatment. Learn more about making treatment decisions. [cancer.net]

We could not identify any correlation between CD20-expression and efficacy of rituximab treatment. [ncbi.nlm.nih.gov]

[…] compared to other leukemias has resulted in difficult production of effective treatments. [en.wikipedia.org]

Prognosis

Although the therapeutic strategy for B-PLL should be determined based on the severity of the disease, RIC with allo-HSCT may be a therapeutic option for indolent B-PLL when the long-term prognosis of patients is markedly poor. [ncbi.nlm.nih.gov]

Sub-classification PLL can transform from CLL. de novo cases of PLL must be distinguished from CLL transformation, as the latter has a worse prognosis. [wiki.clinicalflow.com]

Prognosis can be refined further by grouping CLL patients into three main categories. The patients with the poorest prognosis have unfavorable cytogenetic abnormalities such as del 17p or complex cytogenetics. [cancertherapyadvisor.com]

Etiology

This difference in the spectrum of p53 mutations may reflect the etiologic contribution of distinct exogenous or endogenous factors to B-PLL pathogenesis. [bloodjournal.org]

All blood cultures remained negative for bacterial or fungal etiology. Bronchoalveolar lavage confirmed evidence of PCP as well as CMV by PCR with no evidence of fungal elements. His blood CMV viral titers were over 3.9 × 10 6 copies/ml. [ehoonline.biomedcentral.com]

Epidemiology

10 Codes C91.3 Prolymphocytic Leukemia Corresponding ICD-10-CM Codes (U.S. only) C91.3 Prolymphocytic leukemia of B-cell type (effective October 01, 2015) Diagnostic Exams Immunophenotyping Peripheral blood smear Progression and Transformation None Epidemiology [seer.cancer.gov]

Case Name (click on case name to open) Comments Size Epidemiology B-PLL is an extremely rare disease, comprising approximately 1% of lymphocytic leukemias. [wiki.clinicalflow.com]

About this book Introduction Neoplastic Diseases of the Blood integrates the history, epidemiology, pathology, pathophysiology, and therapeutics of modern neoplastic hematopathology. [doi.org]

About the journal Japanese Journal of Clinical Oncology is an online only (from Jan 2018), multidisciplinary journal for clinical oncologists which publishes high quality articles on medical oncology, clinical trials, radiology, surgery, basic research, epidemiology [jjco.oxfordjournals.org]

Pathophysiology

About this book Introduction Neoplastic Diseases of the Blood integrates the history, epidemiology, pathology, pathophysiology, and therapeutics of modern neoplastic hematopathology. [doi.org]

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

Pathophysiology CLL is a lymphoproliferative disorder that arises from mature B cells and leads to the development of functionally incompetent, clonal malignant cells. [cancertherapyadvisor.com]

Prevention

This “alert” generally prevents other cells from becoming infected. Unfortunately, many viruses can outsmart this protective strategy, and they continue to spread the infection. [primaryimmune.org]

Once in the blood, leukemia cells can spread to other organs, where they can prevent other cells in the body from functioning normally. [cancer.org]

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 prognosis [oncologynurseadvisor.com]

[…] utilization of combinations of chemotherapeutic drugs, it is most often paired with immunotherapy treatments. [10] Targeted Therapy [ edit ] Monoclonal Antibodies [ edit ] A type of targeted therapy that recognizes specific proteins in leukemia cells preventing [en.wikipedia.org]

Similarly, although pentamidine was used to prevent PCP pneumonitis, it was ineffective in this case. Trimethoprim/sulfisoxazole, although may lead to prolonged neutropenia, may be more effective and should be considered. [ehoonline.biomedcentral.com]

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