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PMID- 28395106
DA  - 20170410
DCOM- 20170414
LR  - 20170414
IS  - 1943-7722 (Electronic)
IS  - 0002-9173 (Linking)
VI  - 147
IP  - 2
DP  - 2017 Feb 01
TI  - Primary/Congenital Immunodeficiency: 2015 SH/EAHP Workshop Report-Part 5.
PG  - 204-216
LID - 10.1093/ajcp/aqw215 [doi]
AB  - Objectives: The 2015 Workshop of the Society for Hematopathology/European
      Association for Haematopathology aimed to review primary immunodeficiency and
      related lymphoproliferations. Methods: Primary immunodeficiencies were divided
      into immune dysregulation, DNA repair defects, low immunoglobulins, and combined 
      immunodeficiencies. Results: Autoimmune lymphoproliferative syndrome (ALPS) is a 
      prototypical immune dysregulation-type immunodeficiency, with defects in T-cell
      signaling or apoptosis, expansion of T-cell subsets, and predisposition to
      hemophagocytic lymphohistiocytosis. DNA repair defects directly predispose to
      malignancy. Low immunoglobulin immunodeficiencies such as common variable
      immunodeficiency (CVID) have underlying T-cell repertoire abnormalities
      predisposing to autoimmunity and B-cell lymphoproliferations. The full spectrum
      of B-cell lymphoproliferative disorders occurs in primary immunodeficiency.
      Conclusions: Lymphoproliferations in primary immunodeficiency mirror those in
      other immunodeficiency settings, with monomorphic B- and sometimes T
      lymphoproliferative disorders enriched in DNA repair defects. Distinctive T-cell 
      subset expansions in ALPS, CVID, and related entities can mimic lymphoma, and
      recognition of double-negative T-cell or cytotoxic T-cell expansions is key to
      avoid overdiagnosis.
FAU - Gratzinger, Dita
AU  - Gratzinger D
AD  - From the Stanford University School of Medicine, Stanford, CA.
FAU - Jaffe, Elaine S
AU  - Jaffe ES
AD  - National Institutes of Health, Bethesda, MD.
FAU - Chadburn, Amy
AU  - Chadburn A
AD  - Weill Medical College of Cornell University, New York, NY.
FAU - Chan, John K C
AU  - Chan JK
AD  - Queen Elizabeth Hospital, Kowloon, Hong Kong.
FAU - de Jong, Daphne
AU  - de Jong D
AD  - VU University Medical Center, Amsterdam, the Netherlands.
FAU - Goodlad, John R
AU  - Goodlad JR
AD  - University of Leeds, United Kingdom.
FAU - Said, Jonathan
AU  - Said J
AD  - University of California Los Angeles Medical Center, Los Angeles.
FAU - Natkunam, Yasodha
AU  - Natkunam Y
AD  - From the Stanford University School of Medicine, Stanford, CA.
LA  - eng
PT  - Journal Article
PL  - England
TA  - Am J Clin Pathol
JT  - American journal of clinical pathology
JID - 0370470
SB  - IM
MH  - Education
MH  - Female
MH  - Humans
MH  - Immunologic Deficiency Syndromes/*complications/immunology
MH  - Lymphoproliferative Disorders/*diagnosis/*etiology/*pathology
MH  - Male
OT  - Autoimmune lymphoproliferative syndrome
OT  - Common variable immunodeficiency
OT  - Double negative T cells
OT  - Primary immunodeficiency
OT  - T-cell lymphoma
EDAT- 2017/04/11 06:00
MHDA- 2017/04/15 06:00
CRDT- 2017/04/11 06:00
AID - 3045139 [pii]
AID - 10.1093/ajcp/aqw215 [doi]
PST - ppublish
SO  - Am J Clin Pathol. 2017 Feb 1;147(2):204-216. doi: 10.1093/ajcp/aqw215.

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