IgG4-related sclerosing cholangitis associated with essential thrombocythemia: A case report

Table 1 Main outcomes of laboratory tests at hospitalization

Variable	September 15, 2022	September 23, 2022	September 30, 2022	October 04, 2022	October 09, 2022	October 10, 2022	October 14, 2022	October 18, 2022	October 28, 2022
TBIL in μmol/L	151.79	186.78	156.36	135.41	123.79	99.15	83.87	134.00	53.00
ALP in U/L	895.2	856.2	722.0	837.0	1077.0	856.0	715.0	827.0	745.0
GGT in U/L	NA	393.3	407.0	475.0	709.0	580.0	469.0	889.0	1021.0
PLT as × 109/L	998	760	771	813	910	NA	804	1035	813
D-dimer in g/L	NA	NA	0.39	0.39	0.55	NA	1.45	2.33	NA
PT in s	11.4	NA	11.0	11.1	11.0	NA	10.9	9.9	NA
APTT in s	27.9	NA	42.5	35.6	42.5	NA	31.6	30.6	NA
INR	1.00	NA	0.99	1.00	0.99	NA	0.98	0.89	NA
FIB in g/L	3.68	NA	3.99	3.57	3.99	NA	3.72	3.48	NA
COL/EPI-CT in s	NA	NA	NA	NA	NA	NA	> 292	NA	NA
COL/ADP-CT in s	NA	NA	NA	NA	NA	NA	244	NA	NA
P2Y12-CT in s	NA	NA	NA	NA	NA	NA	136	NA	NA

ALP: Alkaline phosphatase; APTT: Activated partial thromboplastin time; COL/ADP-CT (s): Collagen–adenosine diphosphate closure time(s) (normal range: 62-100s); COL/EPI-CT (s): Collagen–epinephrine closure time (s) (normal range: No drugs, 82-150 s; drugs, 193-300 s); FIB: Fibrinogen; GGT: γ-glutamyl transpeptidase; INR: International normalized ratio; NA: Not available; P2Y12-CT(s): Purinergic receptor P2Y, G protein-coupled 12- closure time (s) (normal range: 106-300s); PLT: Platelet; PT: Prothrombin time; TBIL: Total bilirubin. COL/EPI-CT, COL/ADP-CT, P2Y12-CT are indicators of platelet function, and larger values indicate lower platelet function.

Table 2 Criteria for diagnosis of essential thrombocythemia

Major criteria	Minor criteria
Platelet count ≥ 450000 per mm3	Presence of clonal marker or of evidence of reactive thrombocytosis
Bone marrow biopsy showing proliferation mainly of the megakaryocytic lineage, with increased numbers of enlarged, mature megakaryocytes with hyperlobated nuclei; no substantial increase or left shift in neutrophil granulopoiesis or erythropoiesis; in rare instances, minor (grade 1) increase in reticulin fibers
Criteria for BCR-ABL1-positive chronic myeloid leukemia, polycythemia vera, primary myelofibrosis, or other myeloid neoplasm not met; JAK2 V617F, CALR, or MPL mutation.

Diagnosis requires all major criteria or the first three major criteria plus a minor criterion. CALR: Calreticulin; JAK2: Janus kinase 2; MPL: Myeloproliferative leukemia.