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Aggressive natural killer-cell neoplasm presenting in the marrow: a report of two cases including one with gains of chromosomes 4q and 9p.

Jhuang JY, Clipson A, Hsieh YC, Yang CC, Chang ST, Du MQ, Chuang SS - Diagn Pathol (2015)

Bottom Line: Both patients passed away in a month.One case showed gains of chromosomes 4q and 9p by array comparative genomic hybridization.Our cases demonstrate that lymphoma should be considered in patients with fever of unknown origin and bone marrow aspiration/biopsy should be performed as early diagnosis and novel therapeutic regimens may benefit these patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Anatomic Pathology, Far Eastern Memorial Hospital, New Taipei City, Taiwan.

ABSTRACT
Aggressive nature killer (NK)-cell neoplasm includes aggressive NK-cell leukemia (ANKL) and extranodal NK/T-cell lymphoma (ENKTL), nasal type. ANKL is rare and is characterized by a systemic neoplastic proliferation of NK-cells, usually with a leukemic presentation. ENKTL is a predominantly extranodal lymphoma, occurring mainly in the upper aerodigestive tract. Both are aggressive neoplasms strongly associated with Epstein-Barr virus (EBV). Here we report two patients with aggressive NK-cells neoplasms localized in the bone marrow (BM) who presented as prolonged fever, anemia, and thrombocytopenia. Both were treated initially as infectious disease. Imaging studies revealed splenomegaly without any nodular lesion or lymphadenopathy. BM examination revealed extensive involvement by EBV-positive NK-cells in both cases. Staging workup including nasal examination/biopsy was negative. Both patients passed away in a month. One case showed gains of chromosomes 4q and 9p by array comparative genomic hybridization. Both tumors were diagnostically challenging due to the unusual clinical presentation and absence of leukemic change, tumor mass or lymphadenopathy. Our cases demonstrate that lymphoma should be considered in patients with fever of unknown origin and bone marrow aspiration/biopsy should be performed as early diagnosis and novel therapeutic regimens may benefit these patients.

No MeSH data available.


Related in: MedlinePlus

Case 2. Scanning (a) and medium-powers (b) of the marrow core show extensive coagulative necrosis with scanty viable cells in the right-side end of the core. Even in the high-power view it is difficult to confidently identify the small to medium-sized atypical lymphocytes among the other hematopoietic cells (c). The atypical lymphocytes are highlighted by immunohistostaining with CD3 (d) with irregular nuclear contours and TIA-1 (e). The neoplastic cells are positive for EBV by in situ hybridization (EBER; f and g, low and high-power views, respectively)
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Fig2: Case 2. Scanning (a) and medium-powers (b) of the marrow core show extensive coagulative necrosis with scanty viable cells in the right-side end of the core. Even in the high-power view it is difficult to confidently identify the small to medium-sized atypical lymphocytes among the other hematopoietic cells (c). The atypical lymphocytes are highlighted by immunohistostaining with CD3 (d) with irregular nuclear contours and TIA-1 (e). The neoplastic cells are positive for EBV by in situ hybridization (EBER; f and g, low and high-power views, respectively)

Mentions: Marrow biopsy of Case 2 showed extensive coagulative necrosis with a remaining small proportion of viable cells at the periphery of the marrow core (Fig. 2a and b). Morphologically, it was difficult to identify the atypical lymphoid cells (Fig. 2c). Immunohistochemical stain with CD3 (Fig. 2d) highlighted the small to medium-sized, polymorphous lymphocytes, including those in the necrotic areas. These cells also expressed CD7, CD8, and TIA-1 (Fig. 2e) and were positive for EBER (Fig. 2f and g) but not CD2, CD4, CD5, CD56, βF1 or TCR-γ. Clonality study for TCR-γ chain gene was polyclonal for both cases using our previously described protocols [5]. There was no fresh/frozen tissue for copy number analysis.Fig. 2


Aggressive natural killer-cell neoplasm presenting in the marrow: a report of two cases including one with gains of chromosomes 4q and 9p.

Jhuang JY, Clipson A, Hsieh YC, Yang CC, Chang ST, Du MQ, Chuang SS - Diagn Pathol (2015)

Case 2. Scanning (a) and medium-powers (b) of the marrow core show extensive coagulative necrosis with scanty viable cells in the right-side end of the core. Even in the high-power view it is difficult to confidently identify the small to medium-sized atypical lymphocytes among the other hematopoietic cells (c). The atypical lymphocytes are highlighted by immunohistostaining with CD3 (d) with irregular nuclear contours and TIA-1 (e). The neoplastic cells are positive for EBV by in situ hybridization (EBER; f and g, low and high-power views, respectively)
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4491245&req=5

Fig2: Case 2. Scanning (a) and medium-powers (b) of the marrow core show extensive coagulative necrosis with scanty viable cells in the right-side end of the core. Even in the high-power view it is difficult to confidently identify the small to medium-sized atypical lymphocytes among the other hematopoietic cells (c). The atypical lymphocytes are highlighted by immunohistostaining with CD3 (d) with irregular nuclear contours and TIA-1 (e). The neoplastic cells are positive for EBV by in situ hybridization (EBER; f and g, low and high-power views, respectively)
Mentions: Marrow biopsy of Case 2 showed extensive coagulative necrosis with a remaining small proportion of viable cells at the periphery of the marrow core (Fig. 2a and b). Morphologically, it was difficult to identify the atypical lymphoid cells (Fig. 2c). Immunohistochemical stain with CD3 (Fig. 2d) highlighted the small to medium-sized, polymorphous lymphocytes, including those in the necrotic areas. These cells also expressed CD7, CD8, and TIA-1 (Fig. 2e) and were positive for EBER (Fig. 2f and g) but not CD2, CD4, CD5, CD56, βF1 or TCR-γ. Clonality study for TCR-γ chain gene was polyclonal for both cases using our previously described protocols [5]. There was no fresh/frozen tissue for copy number analysis.Fig. 2

Bottom Line: Both patients passed away in a month.One case showed gains of chromosomes 4q and 9p by array comparative genomic hybridization.Our cases demonstrate that lymphoma should be considered in patients with fever of unknown origin and bone marrow aspiration/biopsy should be performed as early diagnosis and novel therapeutic regimens may benefit these patients.

View Article: PubMed Central - PubMed

Affiliation: Department of Anatomic Pathology, Far Eastern Memorial Hospital, New Taipei City, Taiwan.

ABSTRACT
Aggressive nature killer (NK)-cell neoplasm includes aggressive NK-cell leukemia (ANKL) and extranodal NK/T-cell lymphoma (ENKTL), nasal type. ANKL is rare and is characterized by a systemic neoplastic proliferation of NK-cells, usually with a leukemic presentation. ENKTL is a predominantly extranodal lymphoma, occurring mainly in the upper aerodigestive tract. Both are aggressive neoplasms strongly associated with Epstein-Barr virus (EBV). Here we report two patients with aggressive NK-cells neoplasms localized in the bone marrow (BM) who presented as prolonged fever, anemia, and thrombocytopenia. Both were treated initially as infectious disease. Imaging studies revealed splenomegaly without any nodular lesion or lymphadenopathy. BM examination revealed extensive involvement by EBV-positive NK-cells in both cases. Staging workup including nasal examination/biopsy was negative. Both patients passed away in a month. One case showed gains of chromosomes 4q and 9p by array comparative genomic hybridization. Both tumors were diagnostically challenging due to the unusual clinical presentation and absence of leukemic change, tumor mass or lymphadenopathy. Our cases demonstrate that lymphoma should be considered in patients with fever of unknown origin and bone marrow aspiration/biopsy should be performed as early diagnosis and novel therapeutic regimens may benefit these patients.

No MeSH data available.


Related in: MedlinePlus