9 July 2009 - Case #151
All cases are archived on our website. To view them sorted by case number, diagnosis or category, visit our main Case of the Month page. To subscribe or unsubscribe to Case of the Month or our other email lists, click here.
This case was contributed by Angel Fernandez-Flores, M.D., Ph.D., from Hospital El Bierzo and Clinica Ponferrada, Ponferrada, Spain, for contributing this case.
Case #151
Clinical history:
An 86 year old man was diagnosed 10 months ago with atypical chronic myeloid leukemia, with lack of basophilia. The bone marrow aspirate was suggestive of non-progression to acute leukemia. He currently presented with more than five cutaneous lesions on his chest and back. They were red indurated papules and the largest measured 1 cm in diameter.
Microscopic images:
What is your diagnosis?
Diagnosis: Leukemic vasculitis in the context of leukemia cutis, in a patient with atypical chronic myeloid leukemia
Immunostains:
Discussion:
Chronic myeloid leukemia (CML) is characterized by the t(9;22)(q34;q11) (Philadelphia chromosome), discovered in 1960 by Peter Nowell of the University of Pennsylvania and David Hungerford of the Fox Chase Cancer Center or by the fusion transcript of the ABL (#9q34) and BCR (#22q11) genes (J Natl Cancer Inst 1960;25:85, Cell 1984;36:93).
Atypical CML is a myelodysplastic / myeloproliferative neoplasm that differs from classic CML by (a) the presence of marked granulocytic and multilineage dysplasia, (b) anemia and thrombocytopenia, (c) the lack of basophilia and (d) the lack of a BCR::ABL fusion transcript by cytogenetics or RT-PCR (Eur J Haematol 2009;83:292). Diagnosis requires RT-PCR to rule out a fusion gene, which may be present even with a normal karyotype (Hematol Oncol 2006;24:86). Atypical CML is also JAK2 negative (Leuk Res 2008;32:1931).
Leukemia cutis is the term used to describe neoplastic infiltration of the skin that arises in patients with systemic leukemias (Am J Clin Pathol 2008;129:130, Praxis 2002; 91:1071). Occasionally leukemia cutis precedes systemic leukemia and can be an early diagnostic factor (Clin Exp Dermatol 2004;29;468). Leukemia cutis tends to indicate an unfavorable prognosis, due to aggressive behavior and short survival (eMedicine: Leukemia Cutis [Accessed 29 April 2024], J Am Acad Dermatol 1999;40:966).
The infiltration of leukemic cells into the dermis or blood vessel walls is termed leukemic vasculitis (J Am Acad Dermatol 2009;61:519, Am J Clin Pathol 1997;107:637). It is often present in leukemia cutis (Br J Dermatol 2000;143:773). It is more aggressive than nonvasculitis leukemia cutis and is associated with a poorer prognosis. The differential diagnosis includes paraneoplastic vasculitis, often caused by antibiotics, cytokines or chemotherapeutic agents (Leuk Lymphoma 2000;40:105).
All cases are archived on our website. To view them sorted by case number, diagnosis or category, visit our main Case of the Month page. To subscribe or unsubscribe to Case of the Month or our other email lists, click here.
This case was contributed by Angel Fernandez-Flores, M.D., Ph.D., from Hospital El Bierzo and Clinica Ponferrada, Ponferrada, Spain, for contributing this case.
Website news:
(1) Thanks to Jennifer Stumph, MD, Spectrum Health, for contributing images of schistosomiasis to the Parasitology chapter.
(2) We updated the Soft Tissue Tumors-Part 2 chapter, which includes Fibrohistiocytic and Adipose tissue tumors. This chapter is in our new format, in which each topic is a separate page that is accessed by clicking on the link in the Table of Contents or Index. The pages now load faster, are easier to read with less scrolling, and include thumbnails for most of the 600+ images.
(3) We have started to apply our new format to the Stains chapter, and have extensively updated the topics on BG-8 (useful for mesothelioma) and D2-40 (a marker of lymphatics). For many stain topics, we now include a link to a company that supplies these markers (for example, Covance for these markers).
Visit and follow our Blog to see recent updates to the website.
(1) Thanks to Jennifer Stumph, MD, Spectrum Health, for contributing images of schistosomiasis to the Parasitology chapter.
(2) We updated the Soft Tissue Tumors-Part 2 chapter, which includes Fibrohistiocytic and Adipose tissue tumors. This chapter is in our new format, in which each topic is a separate page that is accessed by clicking on the link in the Table of Contents or Index. The pages now load faster, are easier to read with less scrolling, and include thumbnails for most of the 600+ images.
(3) We have started to apply our new format to the Stains chapter, and have extensively updated the topics on BG-8 (useful for mesothelioma) and D2-40 (a marker of lymphatics). For many stain topics, we now include a link to a company that supplies these markers (for example, Covance for these markers).
Visit and follow our Blog to see recent updates to the website.
Case #151
Clinical history:
An 86 year old man was diagnosed 10 months ago with atypical chronic myeloid leukemia, with lack of basophilia. The bone marrow aspirate was suggestive of non-progression to acute leukemia. He currently presented with more than five cutaneous lesions on his chest and back. They were red indurated papules and the largest measured 1 cm in diameter.
Microscopic images:
What is your diagnosis?
Click here for diagnosis and discussion:
Diagnosis: Leukemic vasculitis in the context of leukemia cutis, in a patient with atypical chronic myeloid leukemia
Immunostains:
CD45 / LCA
Myeloperoxidase
CD34
CD68
Other CD markers
Discussion:
Chronic myeloid leukemia (CML) is characterized by the t(9;22)(q34;q11) (Philadelphia chromosome), discovered in 1960 by Peter Nowell of the University of Pennsylvania and David Hungerford of the Fox Chase Cancer Center or by the fusion transcript of the ABL (#9q34) and BCR (#22q11) genes (J Natl Cancer Inst 1960;25:85, Cell 1984;36:93).
Atypical CML is a myelodysplastic / myeloproliferative neoplasm that differs from classic CML by (a) the presence of marked granulocytic and multilineage dysplasia, (b) anemia and thrombocytopenia, (c) the lack of basophilia and (d) the lack of a BCR::ABL fusion transcript by cytogenetics or RT-PCR (Eur J Haematol 2009;83:292). Diagnosis requires RT-PCR to rule out a fusion gene, which may be present even with a normal karyotype (Hematol Oncol 2006;24:86). Atypical CML is also JAK2 negative (Leuk Res 2008;32:1931).
Leukemia cutis is the term used to describe neoplastic infiltration of the skin that arises in patients with systemic leukemias (Am J Clin Pathol 2008;129:130, Praxis 2002; 91:1071). Occasionally leukemia cutis precedes systemic leukemia and can be an early diagnostic factor (Clin Exp Dermatol 2004;29;468). Leukemia cutis tends to indicate an unfavorable prognosis, due to aggressive behavior and short survival (eMedicine: Leukemia Cutis [Accessed 29 April 2024], J Am Acad Dermatol 1999;40:966).
The infiltration of leukemic cells into the dermis or blood vessel walls is termed leukemic vasculitis (J Am Acad Dermatol 2009;61:519, Am J Clin Pathol 1997;107:637). It is often present in leukemia cutis (Br J Dermatol 2000;143:773). It is more aggressive than nonvasculitis leukemia cutis and is associated with a poorer prognosis. The differential diagnosis includes paraneoplastic vasculitis, often caused by antibiotics, cytokines or chemotherapeutic agents (Leuk Lymphoma 2000;40:105).
