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Characteristics of progressive multifocal leukoencephalopathy in hematological malignancies, With reference to
Emi Yokoyama1, Taisuke Harada2, Tomoyuki Endo3
1Blood Disorders Center, Aiiku Hospital, Sapporo, Japan. chatte_brillante@yahoo.co.jp.
Abstract:
Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection of the central nervous system (CNS) observed in immunocompromised individuals. We retrospectively evaluated the clinical courses of PML in patients with hematological malignancies at North Japan Hematology Study Group institutions, using patients with human immunodeficiency virus (HIV) infection as a reference. Eight PML patients had hematological malignancies and 9 had HIV infection. CD4 + T cell counts were markedly low in patients with hematological malignancies (median 168/μl, range 48.4-330/μl) and did not differ significantly from those in patients with HIV infection (median 28/μl, range 8-225.4/μl). IgG levels were significantly lower in patients with hematological malignancies (median 643.5 mg/dL, range 205-2478 mg/dL) than in patients with with HIV infection (median 1691.5 mg/dL, range 1304-3014 mg/dL) (p < 0.01). Almost all patients with hematological malignancies died after progression of PML (median time from onset to death, 136 days). In contrast, seven patients with HIV infection were alive at the end of follow-up. The incidence of PML in patients with hematological malignancies may increase with the introduction of new antibody drugs and cellular therapies in future. Considering the poor prognosis, greater caution is warranted regarding this serious complication.
Insights
Progressive multifocal leukoencephalopathy (PML) is a rare CNS infection. Patients with hematological malignancies had lower IgG levels and a poorer prognosis than those with HIV, indicating a need for increased vigilance.
Area of Science:
- Neuroimmunology
- Infectious Diseases
- Hematology
Background:
- Progressive multifocal leukoencephalopathy (PML) is a rare, opportunistic central nervous system (CNS) infection affecting immunocompromised individuals.
- PML is associated with JC virus reactivation and can be devastating in immunocompromised hosts.
Purpose of the Study:
- To compare the clinical course and outcomes of PML in patients with hematological malignancies versus those with human immunodeficiency virus (HIV) infection.
- To assess the immunological parameters and prognosis of PML in these distinct patient populations.
Main Methods:
- Retrospective evaluation of clinical data from PML patients at North Japan Hematology Study Group institutions.
- Comparison of CD4+ T cell counts and IgG levels between PML patients with hematological malignancies and those with HIV infection.
- Analysis of survival data and time from PML onset to death.
Main Results:
- Eight PML patients had hematological malignancies, and nine had HIV infection.
- Patients with hematological malignancies had significantly lower IgG levels (median 643.5 mg/dL) compared to HIV patients (median 1691.5 mg/dL) (p < 0.01).
- Nearly all patients with hematological malignancies died within 136 days of PML onset, while most HIV patients survived.
Conclusions:
- PML carries a poor prognosis in patients with hematological malignancies, characterized by lower IgG levels and high mortality.
- The incidence of PML may rise in hematological malignancy patients due to novel therapies.
- Increased caution and monitoring are crucial for managing PML in immunocompromised individuals, particularly those with hematological malignancies.
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