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Published on: October 14, 2016
[Leukemic meningitis in B-cell prolymphocytic leukemia]
Tohru Takahashi1, Yoshie Kazama, Suguru Ishii
1Second Department of Internal Medicine, Tenshi Hospital.
Insights
This case study details an elderly woman diagnosed with B-cell prolymphocytic leukemia (PLL). The aggressive leukemia led to central nervous system infiltration and ultimately, death within six months.
Area of Science:
- Hematology
- Oncology
- Cell Biology
Background:
- B-cell prolymphocytic leukemia (PLL) is a rare, aggressive lymphoid malignancy.
- Elderly patients often present with high white blood cell counts and organomegaly.
Observation:
- An 84-year-old female presented with severe anemia and marked leukocytosis (237,660/microliter) with 93% abnormal lymphoid cells.
- Immunophenotyping confirmed B-cell lineage (CD19+, CD20+, CD22+, IgM+, IgD+).
- Massive splenomegaly was noted, but no lymphadenopathy.
Findings:
- Complex karyotypic abnormalities were identified.
- Leukemic cells infiltrated the cerebrospinal fluid, causing CNS complications like headache and diplopia.
- Treatment with etoposide and intrathecal chemotherapy provided only transient improvement.
Implications:
- B-cell PLL can present with aggressive features and CNS involvement.
- Effective management strategies for advanced B-cell PLL, especially with CNS infiltration, require further investigation.
- This case underscores the poor prognosis associated with CNS involvement in B-cell PLL.
Abstract:
An 84-year-old woman was admitted because of anemia and marked leukocytosis. The white cell count was 237,660/microliter, with 93% abnormal lymphoid cells. The cells had abundant cytoplasm and prominent nucleoli. They were positive for CD 5, 19, 20, 22, 23, HLA-DR, IgM, IgD and kappa chain. Thus, a diagnosis of B-cell PLL was made. Chromosome analysis disclosed a complex karyotypic abnormality. Massive splenomegaly was detected by abdominal computed tomography. No external or internal lymphadenopathy was found. The patient was intermittently treated with etoposide. Although the white cell counts had been suppressed, she refused to take the drug because of side effects. When the white cell count exceeded more than 200,000/microliter again, she developed severe headache, diplopia, nausea, and vomiting. A lumber puncture disclosed infiltration of the prolymphocytes in the cerebrospinal fluid. Though intrathecal chemotherapy alleviated the symptoms and the leukemic cells disappeared, the effects were transient. When the therapy was withheld because of bone marrow suppression, the meningitis recurred and the symptoms progressed. The patient died six months after the initial presentation.
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