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Acute promyelocytic leukemia presenting with central nervous system involvement: a report of 2 cases.

Ji M, Chi HS, Jang S, Park CJ, Lee JH, Seo JJ - Korean J Lab Med (2011)

Bottom Line: BM studies confirmed APL.She has been maintained in the CR status for the past 31 months.Thus, patients with APL must be evaluated for CNS involvement if any neurological symptoms are present at the time of diagnosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea.

ABSTRACT
Central nervous system (CNS) involvement in acute promyelocytic leukemia (APL) is rare, and the presence of CNS symptoms at the time of diagnosis of APL is even rarer. We report 2 cases of APL presenting with CNS involvement. A 43-yr-old woman presented with easy bruising and stuporous mentality. Her complete blood count (CBC) revealed leukocytosis with increased blasts. Bone marrow (BM) analysis was carried out, and the diagnosis of APL was confirmed. This was done by cytogenetic analysis and demonstration of PML-RARα rearrangement by reverse transcriptase PCR in the BM cells. A lumbar puncture was performed to investigate the cause of her stuporous mentality, and her cerebrospinal fluid (CSF) analysis revealed 97% leukemic promyelocytes. Despite systemic and CNS therapy, she died due to septic shock by infection and rapid disease progression only 3 days after her admission. Another patient, a 3-yr-old girl, presented with easy bruising and epistaxis, and her CBC showed pancytopenia with increased blasts. BM studies confirmed APL. Quantitative PCR for PML-RARα in the BM cells revealed a PML-RARα/ABL ratio of 0.33 and CSF analysis revealed 9.5% leukemic promyelocytes (2 of 21 cells). She received induction chemotherapy and intrathecal therapy and achieved complete remission (CR) in the BM and CNS. She has been maintained in the CR status for the past 31 months. Thus, patients with APL must be evaluated for CNS involvement if any neurological symptoms are present at the time of diagnosis.

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Cytospin preparation of the cerebrospinal fluid of Case 1 showing promyelocytes with Auer rods in the cytoplasm. Numerous red blood cells are seen in the background (Wright-Giemsa stain, ×1,000).
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Figure 1: Cytospin preparation of the cerebrospinal fluid of Case 1 showing promyelocytes with Auer rods in the cytoplasm. Numerous red blood cells are seen in the background (Wright-Giemsa stain, ×1,000).

Mentions: A brain CT scan did not show any tumor or CNS hemorrhage. A lumbar puncture was performed to investigate the cause of her stuporous mentality; and cerebrospinal fluid (CSF) analysis revealed a WBC count of 2.2×109/L, red blood cell (RBC) count of 0.16×1012/L, and leukemic promyelocyte proportion of 97% in the bloody background (Fig. 1). Cytospins were prepared from CSF cells using a Shandon Cytospin 3 (Shandon, Astmoore, UK). The loading volume was 5 drops and centrifugation was performed at 700 rpm for 5 min. Next, Wright-Giemsa staining was carried out. The CSF specimen was not subjected to RT-PCR or FISH analysis.


Acute promyelocytic leukemia presenting with central nervous system involvement: a report of 2 cases.

Ji M, Chi HS, Jang S, Park CJ, Lee JH, Seo JJ - Korean J Lab Med (2011)

Cytospin preparation of the cerebrospinal fluid of Case 1 showing promyelocytes with Auer rods in the cytoplasm. Numerous red blood cells are seen in the background (Wright-Giemsa stain, ×1,000).
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3111037&req=5

Figure 1: Cytospin preparation of the cerebrospinal fluid of Case 1 showing promyelocytes with Auer rods in the cytoplasm. Numerous red blood cells are seen in the background (Wright-Giemsa stain, ×1,000).
Mentions: A brain CT scan did not show any tumor or CNS hemorrhage. A lumbar puncture was performed to investigate the cause of her stuporous mentality; and cerebrospinal fluid (CSF) analysis revealed a WBC count of 2.2×109/L, red blood cell (RBC) count of 0.16×1012/L, and leukemic promyelocyte proportion of 97% in the bloody background (Fig. 1). Cytospins were prepared from CSF cells using a Shandon Cytospin 3 (Shandon, Astmoore, UK). The loading volume was 5 drops and centrifugation was performed at 700 rpm for 5 min. Next, Wright-Giemsa staining was carried out. The CSF specimen was not subjected to RT-PCR or FISH analysis.

Bottom Line: BM studies confirmed APL.She has been maintained in the CR status for the past 31 months.Thus, patients with APL must be evaluated for CNS involvement if any neurological symptoms are present at the time of diagnosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Laboratory Medicine, University of Ulsan College of Medicine and Asan Medical Center, Seoul, Korea.

ABSTRACT
Central nervous system (CNS) involvement in acute promyelocytic leukemia (APL) is rare, and the presence of CNS symptoms at the time of diagnosis of APL is even rarer. We report 2 cases of APL presenting with CNS involvement. A 43-yr-old woman presented with easy bruising and stuporous mentality. Her complete blood count (CBC) revealed leukocytosis with increased blasts. Bone marrow (BM) analysis was carried out, and the diagnosis of APL was confirmed. This was done by cytogenetic analysis and demonstration of PML-RARα rearrangement by reverse transcriptase PCR in the BM cells. A lumbar puncture was performed to investigate the cause of her stuporous mentality, and her cerebrospinal fluid (CSF) analysis revealed 97% leukemic promyelocytes. Despite systemic and CNS therapy, she died due to septic shock by infection and rapid disease progression only 3 days after her admission. Another patient, a 3-yr-old girl, presented with easy bruising and epistaxis, and her CBC showed pancytopenia with increased blasts. BM studies confirmed APL. Quantitative PCR for PML-RARα in the BM cells revealed a PML-RARα/ABL ratio of 0.33 and CSF analysis revealed 9.5% leukemic promyelocytes (2 of 21 cells). She received induction chemotherapy and intrathecal therapy and achieved complete remission (CR) in the BM and CNS. She has been maintained in the CR status for the past 31 months. Thus, patients with APL must be evaluated for CNS involvement if any neurological symptoms are present at the time of diagnosis.

Show MeSH
Related in: MedlinePlus