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PAS-positive lymphocyte vacuolization

MedGen UID:
1053941
•Concept ID:
CN377471
•
Finding
HPO: HP:6000639

Definition

Presence of lymphocytes with positive staining for periodic acid-Schiff (PAS) cytoplasmic vacuoles. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPAS-positive lymphocyte vacuolization

Professional guidelines

PubMed

Novel autophagic vacuolar myopathies: Phenotype and genotype features.
Napolitano F, Terracciano C, Bruno G, De Blasiis P, Lombardi L, Gialluisi A, Gianfrancesco F, De Giovanni D, Tummolo A, Di Iorio G, Limongelli G, Esposito T, Melone MAB, Sampaolo S
Neuropathol Appl Neurobiol 2021 Aug;47(5):664-678. Epub 2021 Feb 1 doi: 10.1111/nan.12690. PMID: 33393119

Recent clinical studies

Etiology

Novel autophagic vacuolar myopathies: Phenotype and genotype features.
Napolitano F, Terracciano C, Bruno G, De Blasiis P, Lombardi L, Gialluisi A, Gianfrancesco F, De Giovanni D, Tummolo A, Di Iorio G, Limongelli G, Esposito T, Melone MAB, Sampaolo S
Neuropathol Appl Neurobiol 2021 Aug;47(5):664-678. Epub 2021 Feb 1 doi: 10.1111/nan.12690. PMID: 33393119
Periodic acid Schiff reaction in childhood lymphoblastic leukaemia. The Medical Research Council Working Party on Childhood Leukaemia.
Lilleyman JS, Britton JA, Anderson LM, Richards SM, Bailey CC, Chessells JM
J Clin Pathol 1994 Aug;47(8):689-92. doi: 10.1136/jcp.47.8.689. PMID: 7962616Free PMC Article
Signet ring cell lymphoma. A rare morphologic and functional expression of nodular (follicular) lymphoma.
Kim H, Dorfman RF, Rappaport H
Am J Surg Pathol 1978 Jun;2(2):119-32. PMID: 350062

Diagnosis

Classic infantile-onset Pompe disease with histopathological neurologic findings linked to a novel GAA gene 4 bp deletion: A case study.
Cerón-Rodríguez M, Castillo-García D, Acosta-Rodríguez-Bueno CP, Aguirre-Hernández J, Murillo-Eliosa JR, Valencia-Mayoral P, Escobar-Sánchez A, Salgado-Loza JL
Mol Genet Genomic Med 2022 Jul;10(7):e1957. Epub 2022 May 9 doi: 10.1002/mgg3.1957. PMID: 35532199Free PMC Article
Novel autophagic vacuolar myopathies: Phenotype and genotype features.
Napolitano F, Terracciano C, Bruno G, De Blasiis P, Lombardi L, Gialluisi A, Gianfrancesco F, De Giovanni D, Tummolo A, Di Iorio G, Limongelli G, Esposito T, Melone MAB, Sampaolo S
Neuropathol Appl Neurobiol 2021 Aug;47(5):664-678. Epub 2021 Feb 1 doi: 10.1111/nan.12690. PMID: 33393119
Vacuolated PAS-positive lymphocytes as an hallmark of Pompe disease and other myopathies related to impaired autophagy.
Pascarella A, Terracciano C, Farina O, Lombardi L, Esposito T, Napolitano F, Franzese G, Panella G, Tuccillo F, la Marca G, Bernardini S, Boffo S, Giordano A, Di Iorio G, Melone MAB, Sampaolo S
J Cell Physiol 2018 Aug;233(8):5829-5837. Epub 2018 Feb 22 doi: 10.1002/jcp.26365. PMID: 29215735
Acute atherosis in vacuum suction biopsies of decidua basalis: An evidence based research definition.
Alnaes-Katjavivi P, Lyall F, Roald B, Redman CW, Staff AC
Placenta 2016 Jan;37:26-33. Epub 2015 Nov 11 doi: 10.1016/j.placenta.2015.10.020. PMID: 26608629
PAS-positive lymphocyte vacuoles can be used as diagnostic screening test for Pompe disease.
Hagemans ML, Stigter RL, van Capelle CI, van der Beek NA, Winkel LP, van Vliet L, Hop WC, Reuser AJ, Beishuizen A, van der Ploeg AT
J Inherit Metab Dis 2010 Apr;33(2):133-9. Epub 2010 Jan 27 doi: 10.1007/s10545-009-9027-4. PMID: 20107902Free PMC Article

Therapy

PAS-positive lymphocyte vacuoles can be used as diagnostic screening test for Pompe disease.
Hagemans ML, Stigter RL, van Capelle CI, van der Beek NA, Winkel LP, van Vliet L, Hop WC, Reuser AJ, Beishuizen A, van der Ploeg AT
J Inherit Metab Dis 2010 Apr;33(2):133-9. Epub 2010 Jan 27 doi: 10.1007/s10545-009-9027-4. PMID: 20107902Free PMC Article
Periodic acid Schiff reaction in childhood lymphoblastic leukaemia. The Medical Research Council Working Party on Childhood Leukaemia.
Lilleyman JS, Britton JA, Anderson LM, Richards SM, Bailey CC, Chessells JM
J Clin Pathol 1994 Aug;47(8):689-92. doi: 10.1136/jcp.47.8.689. PMID: 7962616Free PMC Article

Prognosis

Periodic acid Schiff reaction in childhood lymphoblastic leukaemia. The Medical Research Council Working Party on Childhood Leukaemia.
Lilleyman JS, Britton JA, Anderson LM, Richards SM, Bailey CC, Chessells JM
J Clin Pathol 1994 Aug;47(8):689-92. doi: 10.1136/jcp.47.8.689. PMID: 7962616Free PMC Article

Clinical prediction guides

PAS-positive lymphocyte vacuoles can be used as diagnostic screening test for Pompe disease.
Hagemans ML, Stigter RL, van Capelle CI, van der Beek NA, Winkel LP, van Vliet L, Hop WC, Reuser AJ, Beishuizen A, van der Ploeg AT
J Inherit Metab Dis 2010 Apr;33(2):133-9. Epub 2010 Jan 27 doi: 10.1007/s10545-009-9027-4. PMID: 20107902Free PMC Article
Molecular biological ontogenesis of the thymic reticulo-epithelial cell network during the organization of the cellular microenvironment.
Bodey B, Bodey B Jr, Siegel SE, Kaiser HE
In Vivo 1999 May-Jun;13(3):267-94. PMID: 10459506
Periodic acid Schiff reaction in childhood lymphoblastic leukaemia. The Medical Research Council Working Party on Childhood Leukaemia.
Lilleyman JS, Britton JA, Anderson LM, Richards SM, Bailey CC, Chessells JM
J Clin Pathol 1994 Aug;47(8):689-92. doi: 10.1136/jcp.47.8.689. PMID: 7962616Free PMC Article
Immunoglobulin inclusions in non-Hodgkin's lymphomas.
van den Tweel JG, Taylor CR, Parker JW, Lukes RJ
Am J Clin Pathol 1978 Mar;69(3):306-13. doi: 10.1093/ajcp/69.1.306. PMID: 205130
The pathology of type II skeletal muscle glycogenosis. A light and electron-microscopic study.
Hudgson P, Fulthorpe JJ
J Pathol 1975 Jul;116(3):139-47. doi: 10.1002/path.1711160303. PMID: 172619

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