Table 2.
Literature review of association between ANCA and neutropenia
| Reference | Attributed Cause |
Subject Age, Sex |
Degree of Neutropenia |
ANCA (IF) | ANCA (ELISA) |
Signs of Vasculitis |
Antineutrophil Membrane Antibodies |
ANA | Other |
|---|---|---|---|---|---|---|---|---|---|
| Suspected Drug-Induced | |||||||||
|
| |||||||||
| Sera 2000 (6) | Propylthiouriacil | 46,F | ND | p-ANCA | MPO | Purpura | ND | ND | |
| 28,F | ND | p-ANCA | MPO | None | ND | ND | |||
| 33,F | ND | p-ANCA | MPO | None | ND | ND | |||
|
| |||||||||
| Noh 2001 (45) | Propylthiouriacil | 22,F | Mild | p-ANCA | MPO | None | ND | + | |
|
| |||||||||
| Akamizu 2002 (7) | Propylthiouriacil | 45,F | Severe | p-ANCA | MPO,PR3 | None | ND | + | Hypercellular marrow |
|
| |||||||||
| Yamada 2002 (8) | Propylthiouriacil | 13,F | ND | p-ANCA | MPO | Hematuria | + | ND | |
|
| |||||||||
| Farah 2006 (10) | Propylthiouriacil | 70,F | Severe | p-ANCA | ND | Skin-LCV* | ND | ND | |
|
| |||||||||
| Finucane 2008 (37) | Propylthiouriacil | 31,F | Moderate | c-ANCA | PR3 | None | ND | ND | Hypercellular marrow |
|
| |||||||||
| Ozkok 2009 (9) | Propylthiouriacil | 36,F | Moderate | p-ANCA | MPO | Skin-LCV* | ND | − | Splenomegaly Plasmacytosis |
|
| |||||||||
| Kawachi 1995 (11) | Methimazole | 24,F | Moderate | p-ANCA | MPO | LCV* | ND | + | |
|
| |||||||||
| Sangala 2010 (13) | Hydralazine | 55,F | Severe | p-ANCA | MPO | Pauci-immune GN*; Skin-LCV* | ND | + | Hypercellular marrow |
|
| |||||||||
| Ahmed 2008 (12) | Minocycline | 18,M | Mild | c-ANCA | PR3 | None | ND | + | Maturation arrest |
|
| |||||||||
| Knowles 2009 (15) | Cocaine/Levamisole | 4 Cases# | Severe | p-ANCA (2) | ND | ND | ND | ND | |
| c-ANCA (2) | |||||||||
|
| |||||||||
| Bradford 2010 (17) | Cocaine/Levamisole | 39,F | Mild | a-ANCA | HNE | Skin-LCV* | ND | + | LAC |
| 49,F | Severe | a-ANCA | MPO,PR3,HNE | None | ND | + | |||
|
| |||||||||
| Czuchlewski 2010 (18) | Cocaine/Levamisole | 45,F | Severe | ND | ND | None | − | ND | Plasmacytosis |
| 37,M | Severe | None | + | Plasmacytosis | |||||
| 35,F | Severe | None | ND | Plasmacytosis | |||||
|
| |||||||||
| Walsh 2010 (16) | Cocaine/Levamisole | 57,F | Severe | p-ANCA | ND | None | ND | ND | ACA |
| 22,F | Moderate | p-ANCA | ND | Skin-LCV* | ND | ND | ACA | ||
|
| |||||||||
| Barbano 1999 (20) | Levamisole | 8,M | ND | p-ANCA | ND | None | ND | − | ACA, Splenomegaly |
|
| |||||||||
| Underlying Autoimmune Disease | |||||||||
|
| |||||||||
| Juby 1992 (21) | Felty's Syndrome | 11 cases# | Mild-Severe | p-ANCA (4) | ND | ND | ND | ND | Splenomegaly (7) |
| c-ANCA (7) | |||||||||
|
| |||||||||
| Coremans 1993 (22) | Felty's Syndrome | 23 cases# | Mild-Severe | a-ANCA (22) | Lactoferrin (15) MPO (4) Not identified (4) |
ND | ND | + (17) | |
| p-ANCA (1) | − (6) | ||||||||
|
| |||||||||
| Wehbe 2010 (24) | Autoimmune hepatitis | 49,F | Severe | a-ANCA | ND | None | + | ND | Maturation arrest |
|
| |||||||||
| Cuadrado 2009 (25) | Autoimmune hepatitis | 68,F | Severe | p-ANCA | ND | None | − | + | Maturation arrest |
|
| |||||||||
| Hanawa 2010 (26) | Primary Sclerosing Cholangitis | 19,M | Severe | c-ANCA | ND | None | + | + | |
|
| |||||||||
| Jani 2002 (36) | Ulcerative Colitis | 61,F | Moderate | p-ANCA | MPO,PR3 | None | − | + | Splenomegaly |
|
| |||||||||
| Krishnan 1997 (28) | Sjöogren's Syndrome | 52,F | Severe | ND | ND | Skin-LCV | + | + | Maturation arrest |
|
| |||||||||
| Coppo 2004 (27) | Sjöogren's Syndrome | 62,F | Severe | p-ANCA | MPO | None | − | + | Maturation arrest, LGLS |
| 54,F | Severe | p-ANCA | Not Identified | None | − | + | |||
|
| |||||||||
| Present Report | ANCA-Associated Vasculitis | 49,M | Severe | p-ANCA | MPO,PR3 | Skin-LCV*, SN hearing loss, arthritis, epididymitis | + | − | Maturation arrest |
|
| |||||||||
| No Underlying Autoimmune Disease | |||||||||
|
| |||||||||
| Coppo 2004 (27) | Idiopathic | 16,M | Severe | c-ANCA | Not identified | None | + | − | Maturation arrest |
| 46,F | Mild | c-ANCA | PR3 | None | − | + | |||
| 56,F | Severe | p-ANCA | MPO,HNE | Skin-LCV | − | − | Splenomegaly | ||
| 60,M | Severe | a-ANCA | Not identified | None | + | − | Splenomegaly | ||
| 37,F | Mild | c-ANCA | Not identified | None | + | + | |||
| 44,M | Mild | c-ANCA | Not identified | None | − | − | |||
| 67,F | Severe | p-ANCA | Lactoferrin | None | − | + | Maturation arrest | ||
|
| |||||||||
| Rodrigues 2009 (29) | Cyclic Neutropenia | 38,M | Severe | c-ANCA (2) | 60kDA Protein | None | ND | ND | LGLS (1) |
| 53,F | |||||||||
Key: ND- not described; IF- immunofluorescence; ANCA- antineutrophil cytoplasmic antibodies; ANA- antinuclear antibodies; PTU- propylthiouracil, F- female; M- male; p-ANCA- perinuclear; c-ANCA- cytoplasmic; a-ANCA- atypical, MPO- myeloperoxidase; PR3- proteinase 3; HNE- human neutrophil elastase; LCV – leukocytoclastic vasculitis; GN- glomerulonephritis; SN- sensorineural; Maturation arrest - myeloid lineage on bone marrow biopsy; LGLS- large granular lymphocyte syndrome; LAC- lupus anticoagulant; ACA- anti-cardiolipin antibodies.
Degree of neutropenia: mild – 1000–1500/mm3; moderate- 500–1000/mm3; severe < 500/mm3.
Indicates biopsy-proven finding
Indicates subject level data on age and sex not provided.