Table 1.

Diagnostic work-up and results for chronic immune thrombocytopenia (cITP) and autoimmune pancreatitis (AIP)

Diagnostics	Results (normal values)
Chronic immune thrombocytopenia
Humoral (auto-)immunity
Anti-platelet antibodies	Negative
Nuclear antibodies: ANA, DNA, SM, RNP, RO, LA, SCL, JO, HIST, NUC, ACENA	ANA 1:640 (negative), all others negative
Antiphospholipid antibodies: CARG, CARA, B2GPG, B2GPA, B2GPM	Negative
Mannan binding lectin, complement analyses (C3, C4)	Normal
Immunoglobulins: IgG, IgM, IgA, IgG1, IgG2, IgG3, IgG4	Normal
Cellular immunity (flow cytometry)
T-cells: CD3, CD4/3, CD8/3, CD4/8, HLA-D3, CD25/3, Ta/b, Tg/d, 45RA/45R0, 45R0/45RA, 4/45RA, 4/45R0, 8/45RA, 8/45R0, 45RA/62L/8, 3/127, 3/132, 3/154, DNT	T cells and subtypes: normal
B-cells: CD19, IgD + /CD27 + , IgD-/CD27 + , CD21	B cells and subtypes: normal
NK-cells: CD56/3	NK-cells: normal
Lymphocyte function testing: CD3, SEA and TT induced proliferation	Normal
Bone marrow failure or myelodysplasia/leukemia
Cytogenetics, diepoxybutan testing	46, XY[10], normal
FLOW	No leukemic cells detectable
FISH-MDS (monosomy 7, trisomy 8, monosomy 5, deletion 5q, deletion 7q)	No MDS typical chromosomal anomalies detectable
Bone marrow analysis (Giemsa staining)	Normal cellularity except for increased number of megakaryocytes, compatible with ITP
Infection diseases
Virus nucleic acid testing via PCR in bone marrow/plasma: CMV, PVB19, AdV A/B/C/D/E/F/G, EBV	Negative
Helicobacter pylori (PCR stool)	Negative
HIV serology	Negative
Hepatitis serology: anti-HAV IgG, anti-HAV IgM, HBsAg, anti-HBc IgG, anti-HBc IgM	All negative except for anti-HAV IgG 4 IU/l (negative) and anti-HbSAg > 1000 IU/l
Other autoimmune diseases
Thyroid hormones (TSH, fT4)	Normal
Celiac disease serology	Normal
Autoimmune pancreatitis
Pancreas function testing: serum or stool analyses	Amylase 395 U/L (28–100 U/L), lipase 1262 U/L (7–39 U/L), HbA1c 5,4% (4–6%), OGTT normal, elastase < 50 µg/g stool
Liver function testing: serum analyses	GGT 773 U/L (< 52 U/L), ALT 453 U/L (0–31 U/L), AST 247 U/L (0–34 U/L), ALP 947 U/L (< 390 U/l), TBIL 3.1 mg/dl (0–1 mg/dl), BC 2,76 mg/dl (0–0.25 mg/dl), TP 58,7 g/L (60–80 g/L), PRALB 19 mg/dl (12–42 mg/dl)
Tumor marker: Serum analyses	CA 19–9 30.1 kU/L (0–27 kU/L), CEA 0.8 µg/L (0–3.8 µg/L), NSE 19.6 µg/L (0–16.3 µg/L)
Immunology testing: immunoglobulins: IgG, IgM, IgA, IgG1, IgG2, IgG3, IgG4	IgG 548 mg/dl (698–1194 mg/dl), all others normal
Autoantibodies: ANA, DNA, NUC, ENA subsets (RO, LA, SCL-70, SM, RNP, Jo-1, centromer B, c-ANCA, p-ANCA, X-ANCA, smooth muscle, mitochondria, parietal cells, LKM, CARG, CARA, B2GPG, B2GPA, B2GPM, AMA-M2, SP-100, GP210, LC1, SLA	ANA 1:160 (negative), all others negative
Imaging: abdominal ultrasonography	Slightly enlarged liver with normal tissue echogenicity, dilatation of the intra- and extra-hepatobiliary ducts, and a hypoechoic and enlarged pancreatic head
Magnetic resonance cholangiopancreatography	Abrupt termination of the dilated common bile and pancreatic ducts caused by a pancreatic ‘head mass’
Endoscopic ultrasound (EUS)-guided core biopsy-histopathology: HE and immunological staining	Marked fibrosis, lymphoplasmacytic infiltration, and destruction of pancreatic ducts without an increased number of IgG4-positive plasma cells

ACENA anti-centromere antibodies, AdV adenovirus, ALP alkaline phosphatase, ALT alanin-aminotransferase, AMA-M2: anti-mitochondrial antibodies M2, ANA anti-nuclear antibodies, ANCA anti-neutrophil cytoplasmic antibodies, anti-HAV anti-hepatitis A virus antibodies, anti-HBc anti-hepatitis B core antigen antibodies, AST aspartate transaminase, BC conjugated bilirubin, B2GPA beta-2-glycoprotein-1 IgA antibodies, B2GPG beta-2-glycoprotein-1 IgG antibodies, B2GPM beta-2-glycoprotein-1 IgM antibodies, c-ANCA cytoplasmic ANCA, CARA: anti-cardiolipin IgA antibodies, CARG anti-cardiolipin IgG antibodies, CA19-9 cancer antigen 19–9, CD3 CD3 positive T-lymphocytes, CD4/3 CD4 positive T-cell subsets, CD4/8 CD4/CD8 ratio, CD8/3 CD8 positive T-cell subsets, CD19 B-lymphocytes, CD21 CD21 positive B-cell subsets, CD25/3 activated CD25 positive T-cell subsets, CD56/3 CD56 positive CD3 negative NK-cell subsets, CEA carcinoembryonic antigen, CMV cytomegalovirus, DNT TCR alpha/beta positive CD4 negative CD8 negative T-lymphocytes, DNA native/double-stranded deoxyribonucleic acid antibodies, EBV Epstein–Barr virus, ENA extractable nuclear antigens, FISH-MDS fluorescence in situ hybridization-myelodysplastic syndrome, FLOW flow cytometry, fT4 free thyroxine, GGT gamma-glutamyl transpeptidase, GP210 anti-glycoprotein-210 antibodies, HbA1c hemoglobin A1c, HBsAg hepatitis B surface antigen, HE hematoxylin and eosin, HLA-D3 activated HLA-D positive T-cell subsets, HIST anti-histone antibodies, HIV human immunodeficiency virus, IgD+/CD27+ IgD positive CD27 positive memory B-cell subsets, IgD-/CD27+ IgD negative CD27 positive memory B-cell subsets, JO anti Jo-1 antibodies, LA anti-La antibodies, LC1 anti-liver cytosol antibodies type 1, LKM anti–liver-kidney microsomal antibodies, NUC anti-nucleosome antibodies, NSE neuron specific enolase, OGTT oral glucose tolerance test, p-ANCA perinuclear ANCA, PCR polymerase chain reaction, PRALB prealbumin, PVB19 parvovirus B19, RNP anti-nuclear ribonucleoprotein antibodies, RO anti-Ro-antibodies, SCL anti-Scl-70 antibodies, SEA staphylococcus enterotoxin a, SLA anti-soluble liver antigen antibodies, SM anti-Smith antibodies, SP-100 anti-sp100 antibodies, Ta/b TCR alpha/beta positive T-lymphocytes, TBIL total bilirubin, Tg/d TCR gamma/delta positive T-lymphocytes, TP total protein, TSH thyroid stimulating hormone, TT tetanus toxoid, X-ANCA atypical ANCA, 3/127 IL-7R alpha-chain positive T-lymphocytes, 3/132 common gamma-chain positive T-lymphocytes, 3/154 CD40L positive T-lymphocytes, 4/45RA CD4 positive naive T-cell subsets, 4/45R0 CD4 positive memory T-cell subsets, 8/45RA CD8 positive naive T-cell subsets, 8/45R0 CD8 positive memory T-cell subsets, 45RA/45R0 CD45RA positive naive T-lymphocytes, 45RA/62L/8 CD45RA positive CD62L positive CD8 positive naive T-lymphocytes, 45R0/45RA CD45R0 positive memory T-lymphocytes