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.

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.

Related Concept Videos

Cryptococcal Meningitis01:27

Cryptococcal Meningitis

Cryptococcal meningitis is a life-threatening opportunistic infection predominantly associated with HIV/AIDS, accounting for over 100,000 deaths annually worldwide. However, it also affects individuals with other forms of immunosuppression, including those undergoing immunosuppressive therapy, organ transplant recipients, patients with innate immunodeficiencies, and individuals with hematological disorders. The infection is caused mainly by Cryptococcus neoformans and Cryptococcus gattii,...
Encephalitis l: Introduction01:19

Encephalitis l: Introduction

Encephalitis is inflammation of the brain parenchyma, most often due to infections or autoimmune processes. It presents with neuropsychiatric features such as fever, altered mental status, behavioral changes, cognitive dysfunction, seizures, focal deficits, and sometimes autonomic instability. In some cases, the meninges are also involved, resulting in meningoencephalitis.Infectious CausesInfectious encephalitis is most commonly viral but can also result from bacterial, fungal, or parasitic...
Encephalitis ll: Pathophysiology01:26

Encephalitis ll: Pathophysiology

Encephalitis is inflammation of the brain parenchyma caused by direct viral invasion or immune-mediated mechanisms triggered by infections or tumors. Both processes lead to neuronal injury, disrupted neurotransmission, and diverse neurological symptoms, often with overlapping clinical and pathological features.Autoimmune EncephalitisIn autoimmune encephalitis, antibodies target neuronal antigens on cell surfaces, synapses, or within neurons. A key example is anti-NMDAR encephalitis, which can...
Malaria01:29

Malaria

Malaria pathogenesis in humans reflects a delicate interplay between parasite biology and host response. Clinical illness reflects a host’s immune response to the parasite’s asexual replication cycle, which is often asymptomatic in individuals with partial immunity. From the parasite's perspective, transmission between mosquito and human with minimal host pathology is evolutionarily advantageous. Among the six Plasmodium species infecting humans, P. falciparum and P. vivax dominate in global...
Cytomegalovirus Disease01:27

Cytomegalovirus Disease

Cytomegalovirus (CMV) disease is caused by human cytomegalovirus, a double-stranded DNA virus of the Herpesviridae family. While primary CMV infection is often asymptomatic in immunocompetent individuals, the virus can cause severe disease in neonates and immunocompromised patients. CMV is the most common cause of congenital viral infection in the United States, and a major pathogen in solid organ and hematopoietic stem cell transplant recipients.CMV is transmitted via bodily fluids, sexual...
Tumor Progression02:07

Tumor Progression

Tumor progression is a phenomenon where the pre-formed tumor acquires successive mutations to become clinically more aggressive and malignant. In the 1950s, Foulds first described the stepwise progression of cancer cells through successive stages.
Colon cancer is one of the best-documented examples of tumor progression. Early mutation in the APC gene in colon cells causes a small growth on the colon wall called a polyp. With time, this polyp grows into a benign, pre-cancerous tumor. Further...