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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

In situ hybridization for EBER showing strong signals in the tumor cells (×100)
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Fig4: In situ hybridization for EBER showing strong signals in the tumor cells (×100)

Mentions: Based on immunohistochemical staining, the phenotype of the tumor cells was CD3+, CD43+, CD56+, TIA-1+, CD30+, CD4-, CD5-, CD7-, CD8-, CD20- and CD79a-. Approximately 90 % of the tumor cell nuclei were Ki-67 positive. The vascular endothelial cells were positive for CD31 and CD34, which demonstrated the intravascular nature of the neoplastic cells (Fig. 3a, b, c and d). In situ hybridization for EBER revealed positive signals in virtually all tumor cell nuclei (Fig. 4). DNA extracted from formalin-fixed paraffin-embedded samples was analyzed by polymerase chain reaction (PCR). The molecular study demonstrated a germline configuration of TCR genes without rearrangements (Fig. 5).Fig. 3


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)

In situ hybridization for EBER showing strong signals in the tumor cells (×100)
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Fig4: In situ hybridization for EBER showing strong signals in the tumor cells (×100)
Mentions: Based on immunohistochemical staining, the phenotype of the tumor cells was CD3+, CD43+, CD56+, TIA-1+, CD30+, CD4-, CD5-, CD7-, CD8-, CD20- and CD79a-. Approximately 90 % of the tumor cell nuclei were Ki-67 positive. The vascular endothelial cells were positive for CD31 and CD34, which demonstrated the intravascular nature of the neoplastic cells (Fig. 3a, b, c and d). In situ hybridization for EBER revealed positive signals in virtually all tumor cell nuclei (Fig. 4). DNA extracted from formalin-fixed paraffin-embedded samples was analyzed by polymerase chain reaction (PCR). The molecular study demonstrated a germline configuration of TCR genes without rearrangements (Fig. 5).Fig. 3

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