From www.bloodjournal.org by guest on September 11, 2016. For personal use only.
bloodwork ®
IMAGES
IN HEMATOLOGY
“Russell-like” bodies in Philadelphia chromosome–positive B-lymphoblastic leukemia
c Silvia T. Bunting and Glen Lew, Children’s Healthcare of Atlanta
A
16-year-old girl presented with migratory bone pain. Her complete blood count revealed leukocytosis (24 190/mL) with circulating blasts. Bone marrow aspiration revealed 85% blasts, and immunophenotyping confirmed B-lymphoblastic leukemia (common acute lymphoblastic leukemia). Blast morphology was unusual, with a single large intracytoplasmic ground-glass inclusion displacing the nuclei (panel A). Immunohistochemical stains showed the inclusions were negative for IgM, k, or l light chains; serum immunoglobulins were normal. Cytogenetic analysis showed t(9;22)(q34;q11.2), and polymerase chain reaction testing confirmed the p190 BCR-ABL1 breakpoint. Electron microscopy showed nonspecific granular and somewhat fibrillar material in the inclusions, similar to Russell bodies (panel B). Despite the addition of dasatinib, her response to induction chemotherapy was poor, with 8% residual blasts. She did attain remission with subsequent chemotherapy plus a tyrosine kinase inhibitor, but had persistent minimal residual disease. She subsequently underwent allogeneic stem cell transplantation using 4/6 HLA-matched unrelated cord blood. She had multiple complications including severe graft-versus-host disease (GVHD) and ultimately died in remission 13 months after transplantation due to complications from chronic GVHD. Russell bodies are well described in multiple myeloma and some lymphomas but rarely have been described in B-lymphoblastic leukemia. It is unclear whether this unusual blast morphology contributed to the patient’s poor initial response and ultimate outcome.
For additional images, visit the ASH IMAGE BANK, a reference and teaching tool that is continually updated with new atlas and case study images. For more information visit http://imagebank.hematology.org. © 2014 by The American Society of Hematology 2448
BLOOD, 17 APRIL 2014 x VOLUME 123, NUMBER 16
From www.bloodjournal.org by guest on September 11, 2016. For personal use only.
2014 123: 2448 doi:10.1182/blood-2014-01-547513
''Russell-like'' bodies in Philadelphia chromosome−positive B-lymphoblastic leukemia Silvia T. Bunting and Glen Lew
Updated information and services can be found at: http://www.bloodjournal.org/content/123/16/2448.full.html Articles on similar topics can be found in the following Blood collections BloodWork (465 articles) Free Research Articles (4041 articles) Lymphoid Neoplasia (2370 articles) Information about reproducing this article in parts or in its entirety may be found online at: http://www.bloodjournal.org/site/misc/rights.xhtml#repub_requests Information about ordering reprints may be found online at: http://www.bloodjournal.org/site/misc/rights.xhtml#reprints Information about subscriptions and ASH membership may be found online at: http://www.bloodjournal.org/site/subscriptions/index.xhtml
Blood (print ISSN 0006-4971, online ISSN 1528-0020), is published weekly by the American Society of Hematology, 2021 L St, NW, Suite 900, Washington DC 20036. Copyright 2011 by The American Society of Hematology; all rights reserved.
bloodwork ®
IMAGES
IN HEMATOLOGY
“Russell-like” bodies in Philadelphia chromosome–positive B-lymphoblastic leukemia
c Silvia T. Bunting and Glen Lew, Children’s Healthcare of Atlanta
A
16-year-old girl presented with migratory bone pain. Her complete blood count revealed leukocytosis (24 190/mL) with circulating blasts. Bone marrow aspiration revealed 85% blasts, and immunophenotyping confirmed B-lymphoblastic leukemia (common acute lymphoblastic leukemia). Blast morphology was unusual, with a single large intracytoplasmic ground-glass inclusion displacing the nuclei (panel A). Immunohistochemical stains showed the inclusions were negative for IgM, k, or l light chains; serum immunoglobulins were normal. Cytogenetic analysis showed t(9;22)(q34;q11.2), and polymerase chain reaction testing confirmed the p190 BCR-ABL1 breakpoint. Electron microscopy showed nonspecific granular and somewhat fibrillar material in the inclusions, similar to Russell bodies (panel B). Despite the addition of dasatinib, her response to induction chemotherapy was poor, with 8% residual blasts. She did attain remission with subsequent chemotherapy plus a tyrosine kinase inhibitor, but had persistent minimal residual disease. She subsequently underwent allogeneic stem cell transplantation using 4/6 HLA-matched unrelated cord blood. She had multiple complications including severe graft-versus-host disease (GVHD) and ultimately died in remission 13 months after transplantation due to complications from chronic GVHD. Russell bodies are well described in multiple myeloma and some lymphomas but rarely have been described in B-lymphoblastic leukemia. It is unclear whether this unusual blast morphology contributed to the patient’s poor initial response and ultimate outcome.
For additional images, visit the ASH IMAGE BANK, a reference and teaching tool that is continually updated with new atlas and case study images. For more information visit http://imagebank.hematology.org. © 2014 by The American Society of Hematology 2448
BLOOD, 17 APRIL 2014 x VOLUME 123, NUMBER 16
From www.bloodjournal.org by guest on September 11, 2016. For personal use only.
2014 123: 2448 doi:10.1182/blood-2014-01-547513
''Russell-like'' bodies in Philadelphia chromosome−positive B-lymphoblastic leukemia Silvia T. Bunting and Glen Lew
Updated information and services can be found at: http://www.bloodjournal.org/content/123/16/2448.full.html Articles on similar topics can be found in the following Blood collections BloodWork (465 articles) Free Research Articles (4041 articles) Lymphoid Neoplasia (2370 articles) Information about reproducing this article in parts or in its entirety may be found online at: http://www.bloodjournal.org/site/misc/rights.xhtml#repub_requests Information about ordering reprints may be found online at: http://www.bloodjournal.org/site/misc/rights.xhtml#reprints Information about subscriptions and ASH membership may be found online at: http://www.bloodjournal.org/site/subscriptions/index.xhtml
Blood (print ISSN 0006-4971, online ISSN 1528-0020), is published weekly by the American Society of Hematology, 2021 L St, NW, Suite 900, Washington DC 20036. Copyright 2011 by The American Society of Hematology; all rights reserved.
