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Published on: June 22, 2016
Polymorphic reticulosis is a neoplasm of large granular lymphocytes with CD3+ phenotype
Insights
Nasal T-cell lymphoma (NTL), also known as polymorphic reticulosis, is a proliferation of large granular lymphocytes (LGL) with a CD3+ phenotype. This study examined the morphology and characteristics of these cells in patients with lethal midline granuloma (LMG).
Area of Science:
- Pathology
- Immunology
- Oncology
Background:
- Polymorphic reticulosis, a form of lethal midline granuloma (LMG), was previously termed nasal T-cell lymphoma (NTL) due to T-lymphocyte marker reactivity.
- Emerging evidence suggested NTL might originate from natural killer (NK) cells, which share morphological traits with large granular lymphocytes (LGL).
Purpose of the Study:
- To investigate whether NTL-LMG exhibits the characteristic large granular lymphocyte (LGL) morphology.
- To further characterize the cellular phenotype and viral associations in NTL-LMG.
Main Methods:
- Analysis of lymph node smears, peripheral blood, and cutaneous lesions from four NTL-LMG patients.
- Immunohistochemical staining for cell markers, electron microscopy (EM) for morphology, and Epstein-Barr virus (EBV) detection via PCR and in situ hybridization.
Main Results:
- Patients presented with upper respiratory tract necrotic and granulomatous lesions.
- Histology revealed atypical cells with CD3, CD43, CD45RO, and OPD4 reactivity.
- Electron microscopy confirmed LGL morphology with cytoplasmic granules; EBV was detected in one patient.
Conclusions:
- Nasal T-cell lymphoma-lethal midline granuloma (NTL-LMG), or polymorphic reticulosis, represents a proliferation of large granular lymphocytes (LGL).
- These LGLs possess a CD3+ phenotype, clarifying the cellular origin of this condition.
Background:
Polymorphic reticulosis, a type of lethal midline granuloma (LMG), has been referred to as nasal T-cell lymphoma (NTL) because of its proliferating cells' positive reactivity to anti-T-lymphocyte antibodies. Recently, several studies have suggested that proliferating cells in NTL may be natural killer (NK) in nature. NK cells and human nonmajor histocompatibility-restricted cytotoxic T-lymphocytes have the morphology of large granular lymphocytes (LGL) (i.e., a high cytoplasmic:nuclear ratio and cytoplasmic granules). Whether NTL-LMG possesses an LGL morphology is examined in this study.
Methods:
Two lymph node smears, peripheral blood showing a leukemic picture, and an electron microscope (EM) examination of a cutaneous lesion, respectively, were obtained from four patients with NTL-LMG. Immunohistochemical examination of the proliferating cells and of the Epstein-Barr virus (EBV) genome by both polymerase chain reaction and in situ hybridization also were performed.
Results:
All patients presented with necrotic and granulomatous lesions in the upper respiratory tract. Histology showed polymorphous cellular infiltrates containing large atypical cells with positive reaction to CD3 (three patients), CD43 (two patients), CD45RO (two patients), and OPD4 (one patient). Imprint smears revealed azurophilic large membrane-delimited granules in an ample cytoplasm, which was confirmed by EM. The presence of the EBV genome in the tumor cells was observed in one patient.
Conclusion:
The current findings showed that NTL-LMG or polymorphic reticulosis is a proliferation of LGL with a CD3+ phenotype.
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