Gamma heavy chain disease: a retrospective analysis of 6 cases

Table 1 Clinical characteristics, laboratory tests and underlying diseases of 6 patients with gamma heavy chain disease

Patient	Sex	Age (yr)	Associated autoimmune disease	SPEP (g/L)	Hepatomegaly	Splenomegaly	Lymphadenopathy	Bone marrow involvement	Underlying lymphoid neoplasms at diagnosis	Treatment	Outcome
1	Male	40	AIHA	-	+	+	+	+	Small B-cell lymphoma	CHOP	Died of progressive lymphoma 20 months after diagnosis
2	Female	60	RA	-	-	-	--	-	-	W&W	Stable at 52-month follow-up
3	Female	70	RA	20.2	+	+	+	+	B-cell lymphoma, unclassified	CyBorD	Partial response, relapse with DLBCL transformation; treatment with RCHOP regimen, partial response
4	Female	61	SS	-	-	-	-	-	-	W&W	Stable at 42-month follow-up
5	Female	54	SS	8.3	-	-	+	-		W&W	Progression with DLBCL 19 months after diagnosis; treatment with RCHOP, complete response
6	Female	31	-	9.3	+	+	+	+	T-cell lymphoma, NOS	Methotrexate and dexamethasone	Partial response, lost to follow-up 11 months after diagnosis

Abbreviations: AIHA = autoimmune haemolysis anaemia; RA = rheumatoid arthritis; SS = Sjogren syndrome; SPEP = serum protein electrophoresis; R = rituximab; W&W = watch and wait; CHOP = cyclophosphamide, doxorubicin, vincristine, prednisone; CyBorD = cyclophosphamide, bortezomib, dexamethasone; DLBCL = diffuse large B-cell lymphoma; + =present; - =absent

ISSN: 1750-1172