Role of Chemokine Signalling in the Pathogenesis of Good's Syndrome-Case Reports, Clinical Characterization from Single-Centre PerspectivePrzemyslaw Zdziarski1,4*, Grzegorz Dworacki2, Agnieszka Korzeniowska-Kowal4 and Katarzyna Ziemnicka3
- *Corresponding Author:
- Przemyslaw Zdziarski
Department of Clinical Immuno-oncology
Lower Silesian Center P.O Box 1818 50-385
Fax: +48-61 8547173
E-mail: [email protected]
Received date: June 26, 2016; Accepted date: August 11, 2016; Published date: August 16, 2016
Citation: Zdziarski P, Dworacki G, Kowal AK, Ziemnicka K (2016) Role of Chemokine Signalling in the Pathogenesis of Good’s Syndrome-Case Reports, Clinical Characterization from Single-Centre Perspective. Immunome Res 12:119. doi: 10.4172/1745-7580.10000119
Copyright: © 2016 Zdziarski P, et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
Good’s syndrome (GS), is a rare condition defined as a coexistence of thymoma and hipogammaglobulinaemia. Involvement of central lymphoid organ prompts concept about perturbation in lymphocyte migration and differentiation. Cell homing to bone marrow compartment depends on the CXCR4↔CXCL12 interaction. In this paper we describe two patients with GS-mild and severe form, presenting differences in the expression of CXCR4 on cells in peripheral blood and bone marrow. Patient 1 (mild form): (i) Mild hipogammaglobulinaemia (IgG=150 mg/dL), (ii) Low count of peripheral B cells (14%, 60 cells/μL) and NK (18%, 77cells/μL); high level of T cells (93.3%, 4006 cells/ μL), (iii) Moderate thymic enlargement (9 × 15 cm), (iv) CXCR4 expression in BM 82.6% (20139/μL), 34% CXCR4+CD19+ (8289/μL). Patient 2 (severe form): (i) Severe hipogammaglobulinaemia (IgG=20 mg/dL), (ii) Absence of B and NK cells deficiency (in peripheral blood 0.4% i.e., 10/μL, and 6.17%-47.3/μL respectively), (iii) Severe thymic enlargement (20 × 25 cm) (iv) CXCR4 expression in BM 46.3% (552/μL); 6.1%, CXCR4+CD19+ (174/ μL). Interestingly, the bone marrow of patient with severe form of GS contained more CD10-positive B cells than BM of the patient with mild form (80% vs. 5.88% of B cells), but in former as well as letter a significant proportion of the total CXCR4-positive lymphocytes are negative for B cells marker (comparable: 48.1% and 53.1% respectively). In our patients a tenfold decrease in the CD4-positive γδ T cells (CD4+TCRγδ) counts was observed. Both cases went up fatal due to progression of nasopharyngeal cancer (mild form) and breast cancer (severe form). This data confirm that earliest B cell precursors, pre-pro-B cells and end-stage B cells, plasma cells require CXCR4 ↔ CXCL12 interaction. In contrast in GS weak expression of CXCR4 in marrow precursors is source of B cell differentiation arrest on pro-B cell stage. The low level Nk and CD4+γδ T cells in Good syndrome is the new observation.