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Intravascular NK-cell lymphoma: a case report and review of the literature.

Bi Y, Huo Z, Liang Z, Meng Y, Jia C, Shi X, Song L, Luo Y, Ling Q, Liu T - Diagn Pathol (2015)

Bottom Line: In our case, large neoplastic lymphoid cells are restricted to the lumen of small vessels and exhibit the phenotype of a true NK cell.In addition, some cases including ours carry a poor prognosis as multiple systems or vital organs are involved.It is important to recognize this rare entity to make an appropriate diagnosis and achieve a better understanding regarding the treatment and prognosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. Biyeye81@126.com.

ABSTRACT

Background: Intravascular NK-cell lymphoma (IVNKL) is an extremely rare variant of non-Hodgkin lymphoma. To our knowledge, there are only a few cases reported in the English literature. Here, a case of a 29-year-old male with IVNKL involving the skin of the trunk and 4 extremities and liver is presented. A comprehensive literature review is undertaken to summarize the clinical and pathological features of this disorder.

Findings: In our case, large neoplastic lymphoid cells are restricted to the lumen of small vessels and exhibit the phenotype of a true NK cell. The morphology and immunophenotype, positivity of EBER and NK-cell origin are similar to other IVNKL cases. In addition, some cases including ours carry a poor prognosis as multiple systems or vital organs are involved.

Conclusion: In summary, we report a case of an unusual intravascular lymphoma of NK-cell lineage that displays both clinical and pathological features and compare it with other differential diagnoses. It is important to recognize this rare entity to make an appropriate diagnosis and achieve a better understanding regarding the treatment and prognosis.

No MeSH data available.


Related in: MedlinePlus

a and b Red-violaceous ill-defined and irregular plaques on the lower extremities and trunk
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Fig1: a and b Red-violaceous ill-defined and irregular plaques on the lower extremities and trunk

Mentions: A 29-year-old man presented with erythematous plaques on the thighs and trunk, low fever, intermittent headaches, weight loss and leukopenia. His family history was significant, as his only sister had acute myeloid leukemia (M3) several years previously and was cured by chemotherapy. Physical examination revealed painful, subtle, ill-defined, irregular, blanching erythematous patches on the lower and upper extremities and trunk (Fig. 1a and b) and slight hepatosplenomegaly.Fig. 1


Intravascular NK-cell lymphoma: a case report and review of the literature.

Bi Y, Huo Z, Liang Z, Meng Y, Jia C, Shi X, Song L, Luo Y, Ling Q, Liu T - Diagn Pathol (2015)

a and b Red-violaceous ill-defined and irregular plaques on the lower extremities and trunk
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4488042&req=5

Fig1: a and b Red-violaceous ill-defined and irregular plaques on the lower extremities and trunk
Mentions: A 29-year-old man presented with erythematous plaques on the thighs and trunk, low fever, intermittent headaches, weight loss and leukopenia. His family history was significant, as his only sister had acute myeloid leukemia (M3) several years previously and was cured by chemotherapy. Physical examination revealed painful, subtle, ill-defined, irregular, blanching erythematous patches on the lower and upper extremities and trunk (Fig. 1a and b) and slight hepatosplenomegaly.Fig. 1

Bottom Line: In our case, large neoplastic lymphoid cells are restricted to the lumen of small vessels and exhibit the phenotype of a true NK cell.In addition, some cases including ours carry a poor prognosis as multiple systems or vital organs are involved.It is important to recognize this rare entity to make an appropriate diagnosis and achieve a better understanding regarding the treatment and prognosis.

View Article: PubMed Central - PubMed

Affiliation: Department of Pathology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, No.1 Shuaifuyuan, Wangfujing, DongCheng District, Beijing, 100730, China. Biyeye81@126.com.

ABSTRACT

Background: Intravascular NK-cell lymphoma (IVNKL) is an extremely rare variant of non-Hodgkin lymphoma. To our knowledge, there are only a few cases reported in the English literature. Here, a case of a 29-year-old male with IVNKL involving the skin of the trunk and 4 extremities and liver is presented. A comprehensive literature review is undertaken to summarize the clinical and pathological features of this disorder.

Findings: In our case, large neoplastic lymphoid cells are restricted to the lumen of small vessels and exhibit the phenotype of a true NK cell. The morphology and immunophenotype, positivity of EBER and NK-cell origin are similar to other IVNKL cases. In addition, some cases including ours carry a poor prognosis as multiple systems or vital organs are involved.

Conclusion: In summary, we report a case of an unusual intravascular lymphoma of NK-cell lineage that displays both clinical and pathological features and compare it with other differential diagnoses. It is important to recognize this rare entity to make an appropriate diagnosis and achieve a better understanding regarding the treatment and prognosis.

No MeSH data available.


Related in: MedlinePlus