Korean J Hematol.  2010 Sep;45(3):208-210. 10.5045/kjh.2010.45.3.208.

Long-term survival in a patient with acute promyelocytic leukemia with isolated meningeal relapse

• Kim SH
• Yun J
• Kim HJ
• Kim CK
• Park SK
• Hong DS

• KMID: 2252066
• DOI: http://doi.org/10.5045/kjh.2010.45.3.208

Abstract

The meningeal involvement is rare in acute promyelocytic leukemia. We experienced a 39-year-old woman who achieved complete remission with all-trans retinoic acid, idarubicin and cytarabine therapy. Several months later, she complained of non-specific headache. Her complete blood cell count was normal, but magnetic resonance image of brain revealed focal meningeal enhancement and cerebrospinal fluid showed leukemic promyelocytes and PML/RARA rearrangement. Bone marrow study showed hematologic, cytogenetic and molecular remission. She was treated with intrathecal and systemic chemotherapy and whole brain radiotherapy. The patient has survived for 68 months since the last systemic chemotherapy.

Keyword

APL; Extramedullary involvement; CNS

MeSH Terms

Adult
Blood Cell Count
Bone Marrow
Brain
Cytarabine
Cytogenetics
Female
Granulocyte Precursor Cells
Headache
Humans
Idarubicin
Leukemia, Promyelocytic, Acute
Magnetic Resonance Spectroscopy
Recurrence
Tretinoin
Cytarabine
Idarubicin
Tretinoin

Figure

• 

  Fig. 1 Enhanced T1-weighted magnetic resonance image showing focal meningeal enhancement (arrow) in the right parietal lobe.

• 

  Fig. 2 Microscopic finding of cerebrospinal fluid showing numerous leukemic promyelocytes with prominent nuclear irregularities and variably granulated cytoplasms (Wright stain; ×1,000).

Reference

1. Fenaux P, Chastang C, Chevret S, et al. A randomized comparison of all transretinoic acid (ATRA) followed by chemotherapy and ATRA plus chemotherapy and the role of maintenance therapy in newly diagnosed acute promyelocytic leukemia. Blood. 1999; 94:1192–1200. PMID: 10438706.

2. Wiernik PH, De Bellis R, Muxi P, Dutcher JP. Extramedullary acute promyelocytic leukemia. Cancer. 1996; 78:2510–2514. PMID: 8952559.

3. Bae SH, Ryoo HM, Cho HS, Lee JL, Lee KH, Hyun MS. Meningeal relapse in a Patient with acute promyelocytic leukemia: a case report and review of the literature. J Korean Med Sci. 2004; 19:311–314. PMID: 15082912.

4. Anguita E, Villegas A, González FA, Díaz-Mediavilla J, Lo Coco F. Acute promyelocytic leukemia relapse in the central nervous system during hematologic and molecular remission. Am J Hematol. 1999; 60:170–171. PMID: 9929118.

5. Mesa JR, Espinosa E, Losada R, Hernandez C, Martinez G, Hernandez P. Parotid and central nervous system relapse during complete hematologic remission in acute promyelocytic leukemia. Haematologica. 1999; 84:565–566. PMID: 10366808.

6. Liso V, Specchia G, Pogliani EM, et al. Extramedullary involvement in patients with acute promyelocytic leukemia: a report of seven cases. Cancer. 1998; 83:1522–1528. PMID: 9781945.

7. Akoz AG, Dagdas S, Ozet G, Ceran F, Yilmaz M. Isolated central nervous system relapse during cytologic and molecular hematologic remission in two patients with acute promyelocytic leukemia. Hematology. 2007; 12:419–422. PMID: 17852441.

8. Ko BS, Tang JL, Chen YC, et al. Extramedullary relapse after all-trans retinoic acid treatment in acute promyelocytic leukemia-the occurrence of retinoic acid syndrome is a risk factor. Leukemia. 1999; 13:1406–1408. PMID: 10482992.

9. Martino B, Vincelli I, Marino A, Comis M, Ronco F, Nobile F. Meningeal relapse in a patient with acute promyelocytic leukaemia treated with all-trans retinoic acid. Br J Haematol. 1998; 100:606–607. PMID: 9504652.

10. Marchetti M, Falanga A, Giovanelli S, Oldani E, Barbui T. All-trans retinoic acid increases adhesion to endothelium of the human promyelocytic leukaemia cell line NB4. Br J Haematol. 1996; 93:360–366. PMID: 8639429.

11. Saiki I, Fujii H, Yoneda J, et al. Role of aminopeptidase N (CD13) in tumor-cell invasion and extracellular matrix degradation. Int J Cancer. 1993; 54:137–143. PMID: 8097496.

12. Specchia G, Lo Coco F, Vignetti M, et al. Extramedullary involvement at relapse in acute promyelocytic leukemia patients treated or not with all-trans retinoic acid: a report by the Gruppo Italiano Malattie Ematologiche dell'Adulto. J Clin Oncol. 2001; 19:4023–4028. PMID: 11600603.

13. Wiernik PH, De Bellis R, Muxi P, Dutcher JP. Extramedullary acute promyelocytic leukemia. Cancer. 1996; 78:2510–2514. PMID: 8952559.

14. Blaney SM, Balis FM, Poplack DG. Current pharmacological treatment approaches to central nervous system leukaemia. Drugs. 1991; 41:702–716. PMID: 1712705.

15. Au WY, Tam S, Fong BM, Kwong YL. Determinants of cerebrospinal fluid arsenic concentration in patients with acute promyelocytic leukemia on oral arsenic trioxide therapy. Blood. 2008; 112:3587–3590. PMID: 18703707.