Hepatosplenic T-cell lymphoma in a 47-year-old Crohn’s disease patient on thiopurine monotherapy

Table 1 Results of our patient

A: laboratory findings			B: immunophenotyping of liver and bone marrow biopsy
Analysis (Unit)	Results	Reference value	Immunophenotype	Results	Typical HSTCL[1,3]
White blood cell count (× 109/L)	4.6	4.0-10.0	CD 2 and 3	+	+
Platelet count (× 109/L)	42	150-400	CD 4 and 5	-	-
Hemoglobin (mmol/L)	7.7	8.5-11.0	CD 7	+	+/-
Bilirubin (μmol/L)	175	0-17	CD 8	+/-	-
ALP (IU/L)	265	0-125	CD 16	-	+/-
GGT (IU/L)	154	0-45	CD 56	+	+
ASAT (IU/L)	250	0-34	γδ-T-cell receptor	+	+
ALAT (IU/L)	437	0-44	TIA	-	+
LDH (IU/L)	701	0-247

ALP: Alkaline phosphatase; ASAT: Aspartate transaminase; ALAT: Alanine transaminase; GGT: γ-glutamyl- transferase; LDH: Lactate dehydrogenase; TIA: T-cell restricted intracellular antigen.

Table 2 Number of Crohn’s disease cases with hepatosplenic T-cell lymphoma[7,15]

Age	Combination therapy	Monotherapy	Total
> 35 year	7	0	7
< 35 year	20	11	31
Total	27	11	38

Table 3 Cases of γδ-hepatosplenic T-cell lymphoma in patients with Crohn’s disease on thiopurine monotherapy

Index	Age, sex	Years of thiopurine	Presentation	Treatment	Survival (mo)
		treatment (type)
Index case	47, M	14 (AZT)	HSM, icterus, anemia, thrombocytopenia	Ch. (CHOP)	< 1
Selvaraj et al[7] 2013 (AERS 6751796)	18, M	< 1 (AZT, 6MP)	NS	NS	7
Selvaraj et al[7] 2013 (AERS 7554658)	13, M	NS (6MP)	NS	NS	NS
Fowler et al[18] 2010	19, M	6 (AZT)	SM, leucocytopenia	Ch. (NS) + splenectomy	4
Fowler et al[18] 2010	22, M	8 (6MP)	SM, night sweats, fever, abdominal tenderness	Ch. (NS)	Survival
Ochenrider et al[8] 2010	18, M	5 (6MP)	Fever, night sweats, SM, anemia, thrombocytopenia	Ch. (Pentostatin, ICE) + auto-SCT	7
Humphreys et al[19] 2008	27, F	5 (AZT)	Fever, nights sweats, pancytopenia, HSM	Interferon-α	> 31
Zeidan et al[20] 2007	31, M	6 (6MP)1	Chills, SM, fever, pancytopenia	Ch. (CHOP, cytarabine, ESHAP)	7
Falchook et al[15] 2006	NS	NS (6MP)	SM	Ch. (NS)	NS
Mittal et al[21] 2006	18, M	6 (AZT)	Fever, pancytopenia, HSM	Ch. (IVE, ESHAP, alemtuzumab	NS
				fludarabine) + allo-SCT
Navarro et al[22] 2003	35, M	5.6 (AZT)	Fever, night sweats, HSM, anemia, thrombocytopenia	Ch. (NS) + splenectomy	NS
Lémann et al[23] 1998	NS	4 (AZT)	NS	NS	NS

¹Received one single gift infliximab 51 mo before presentation, therefore considered as TNF-α inhibitor naive. AERS: Adverse Event Reporting System; AZT: Azathioprine; Ch.: Chemotherapy; CHOP: Cyclophosphamide, hydroxydoxorubicin, vincristine and prednisone; EHSAP: Etoposide, methylprednisolone, cytarabin, cisplatin; ICE: Ifosphamide, carboplatin, etoposide; IVE: Ifosphamide, carboplatin and etoposide; NS: Not specified; (H)SM: (hepato)splenomegaly; SCT: Stem cell transplantation; 6MP: 6-mercaptopurine.