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Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence

Year 2016, , 114 - 116, 01.04.2016

Rafet Eren Tayfur Toptaş İşık Kaygusuz Atagündüz Tülin Fıratlı Tuğlular

https://doi.org/10.5472/MMJcr.2902.02

Abstract

A previously healthy 31-year-old female presenting with dyspnea, iron deficiency anemia, pancytopenia, splenomegaly, and abnormal coagulation tests was admitted to the hospital. Hematology consultation additionally revealed that 71% of cells were indicative of acute promyelocytic leukemia (APL) and the patient tested positive for a t(15;17) translocation, confirming APL. All-trans retinoic acid (ATRA) therapy was initiated immediately, but the patient exhibited severe dyspnea. This subsequently resulted in circulatory and respiratory arrest, followed by death. Just after death, fluorescein-labelled proaerolysin (FLAER) revealed a paroxysmal nocturnal hemoglobinuria (PNH) monocyteclone of 82%, confirming the diagnosis of PNH. Leukemia can be derived from non-PNH clones in PNH patients. Catastrophic thromboembolic events that could not be controlled with aggressive anticoagulation in a profoundly thrombocytopenic patient without overt disseminated intravascular coagulation (DIC) may suggest co-existent PNH.Keywords: Leukemia, Promyelocytic, Acute, Hemoglobinuria, Paroxysmal

References

  • Parker CJ. Paroxysmal nocturnal hemoglobinuria. Curr Opin Hematol 2012;19:141-8. doi: 10.1097/MOH.0b013e328351c348
  • Harris JW, Koscick R, Lazarus HM, Eshleman JR, Medof ME. Leukemia arising out of paroxysmal nocturnal hemoglobinuria. Leuk Lymphoma 1999;32:401-26.
  • Levi M, Toh CH, Thachil J, Watson HG. Guidelines for the diagnosis and management of disseminated intravascular coagulation. Br J Haematol 2009;145:24-33. doi: 10.1111/j.13652141.2009.07600.x.
  • Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R, Hillmen P, Luzzatto L, Young N, Kinoshita T, Rosse W, Socié G; International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood 2005;106:3699-709. doi: 10.1182/blood-2005-04-1717
  • Ribeiro R, Rego R. Management of APL in developing countries: epidemiology, challenges and opportunities for international collaboration. Hematology Am Soc Hematol Educ Program 2006:162-8.
  • Douer D. The epidemiology of acute promyelocytic leukemia. Bailliere’s Best Pract Clin Hematol 2003;16:357-67. doi: http://dx.doi.org/10.1016/S1521-6926(03)00065-3
  • Mortazavi Y, Tooze JA, Gordon-Smith EC, Rutherford TR. N-RAS gene mutation in patients with aplastic anemia and aplastic anemia/ paroxysmal nocturnal hemoglobinuria during evolution to clonal disease. Blood 2000;95:646-50.

Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence

Year 2016, , 114 - 116, 01.04.2016

Rafet Eren Tayfur Toptaş İşık Kaygusuz Atagündüz Tülin Fıratlı Tuğlular

https://doi.org/10.5472/MMJcr.2902.02

Abstract

A previously healthy 31-year-old female presenting with dyspnea, iron deficiency anemia, pancytopenia, splenomegaly, and abnormal coagulation tests was admitted to the hospital. Hematology consultation additionally revealed that 71% of cells were indicative of acute promyelocytic leukemia (APL) and the patient tested positive for a t(15;17) translocation, confirming APL. All-trans retinoic acid (ATRA) therapy was initiated immediately, but the patient exhibited severe dyspnea. This subsequently resulted in circulatory and respiratory arrest, followed by death. Just after death, fluorescein-labelled proaerolysin (FLAER) revealed a paroxysmal nocturnal hemoglobinuria (PNH) monocyteclone of 82%, confirming the diagnosis of PNH. Leukemia can be derived from non-PNH clones in PNH patients. Catastrophic thromboembolic events that could not be controlled with aggressive anticoagulation in a profoundly thrombocytopenic patient without overt disseminated intravascular coagulation (DIC) may suggest co-existent PNH.Keywords: Leukemia, Promyelocytic, Acute, Hemoglobinuria, Paroxysmal

Keywords

Lösemi Promyelositik Akut Hemoglobinuri Proksismal

References

  • Parker CJ. Paroxysmal nocturnal hemoglobinuria. Curr Opin Hematol 2012;19:141-8. doi: 10.1097/MOH.0b013e328351c348
  • Harris JW, Koscick R, Lazarus HM, Eshleman JR, Medof ME. Leukemia arising out of paroxysmal nocturnal hemoglobinuria. Leuk Lymphoma 1999;32:401-26.
  • Levi M, Toh CH, Thachil J, Watson HG. Guidelines for the diagnosis and management of disseminated intravascular coagulation. Br J Haematol 2009;145:24-33. doi: 10.1111/j.13652141.2009.07600.x.
  • Parker C, Omine M, Richards S, Nishimura J, Bessler M, Ware R, Hillmen P, Luzzatto L, Young N, Kinoshita T, Rosse W, Socié G; International PNH Interest Group. Diagnosis and management of paroxysmal nocturnal hemoglobinuria. Blood 2005;106:3699-709. doi: 10.1182/blood-2005-04-1717
  • Ribeiro R, Rego R. Management of APL in developing countries: epidemiology, challenges and opportunities for international collaboration. Hematology Am Soc Hematol Educ Program 2006:162-8.
  • Douer D. The epidemiology of acute promyelocytic leukemia. Bailliere’s Best Pract Clin Hematol 2003;16:357-67. doi: http://dx.doi.org/10.1016/S1521-6926(03)00065-3
  • Mortazavi Y, Tooze JA, Gordon-Smith EC, Rutherford TR. N-RAS gene mutation in patients with aplastic anemia and aplastic anemia/ paroxysmal nocturnal hemoglobinuria during evolution to clonal disease. Blood 2000;95:646-50.
There are 7 citations in total.

Details

Subjects Clinical Sciences
Other ID JA95GC95DM
Journal Section Review Makaleler
Authors

Rafet Eren This is me

Tayfur Toptaş This is me

İşık Kaygusuz Atagündüz This is me

Tülin Fıratlı Tuğlular This is me

Publication Date April 1, 2016
Published in Issue Year 2016

Cite

APA Eren, R., Toptaş, T., Atagündüz, İ. K., Tuğlular, T. F. (2016). Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence. Marmara Medical Journal, 29(2), 114-116. https://doi.org/10.5472/MMJcr.2902.02
AMA Eren R, Toptaş T, Atagündüz İK, Tuğlular TF. Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence. Marmara Med J. April 2016;29(2):114-116. doi:10.5472/MMJcr.2902.02
Chicago Eren, Rafet, Tayfur Toptaş, İşık Kaygusuz Atagündüz, and Tülin Fıratlı Tuğlular. “Acute Promyelocytic Leukemia Evolving from Paroxysmal Nocturnal Hemoglobinuria: A Rare Occurrence”. Marmara Medical Journal 29, no. 2 (April 2016): 114-16. https://doi.org/10.5472/MMJcr.2902.02.
EndNote Eren R, Toptaş T, Atagündüz İK, Tuğlular TF (April 1, 2016) Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence. Marmara Medical Journal 29 2 114–116.
IEEE R. Eren, T. Toptaş, İ. K. Atagündüz, and T. F. Tuğlular, “Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence”, Marmara Med J, vol. 29, no. 2, pp. 114–116, 2016, doi: 10.5472/MMJcr.2902.02.
ISNAD Eren, Rafet et al. “Acute Promyelocytic Leukemia Evolving from Paroxysmal Nocturnal Hemoglobinuria: A Rare Occurrence”. Marmara Medical Journal 29/2 (April 2016), 114-116. https://doi.org/10.5472/MMJcr.2902.02.
JAMA Eren R, Toptaş T, Atagündüz İK, Tuğlular TF. Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence. Marmara Med J. 2016;29:114–116.
MLA Eren, Rafet et al. “Acute Promyelocytic Leukemia Evolving from Paroxysmal Nocturnal Hemoglobinuria: A Rare Occurrence”. Marmara Medical Journal, vol. 29, no. 2, 2016, pp. 114-6, doi:10.5472/MMJcr.2902.02.
Vancouver Eren R, Toptaş T, Atagündüz İK, Tuğlular TF. Acute promyelocytic leukemia evolving from paroxysmal nocturnal hemoglobinuria: A rare occurrence. Marmara Med J. 2016;29(2):114-6.