Limits...
Thymus and mediastinal node involvement in childhood Langerhans cell histiocytosis: long-term follow-up from the French national cohort.

Ducassou S, Seyrig F, Thomas C, Lambilliotte A, Marec-Berard P, Berger C, Plat G, Brugiere L, Ouache M, Barkaoui M, Armari-Alla C, Lutz P, Leverger G, Rialland X, Mansuy L, Pacquement H, Jeziorski E, Gandemer V, Chalard F, Chateil JF, Tazi A, Emile JF, Donadieu J, Investigators of the French LCH Study Gro - Pediatr Blood Cancer (2013)

Bottom Line: Thirteen cases (35%) were diagnosed because of MI-related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10).The overall 5-year survival was 87.1%, and immunodeficiency was not detected as a sequel.MI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications.

View Article: PubMed Central - PubMed

Affiliation: Service D'hémato Oncologie Pédiatrique, CHU de Strasbourg, Strasbourg, France; Service D'hémato Oncologie Pédiatrique, CHU de Bordeaux, Bordeaux, France.

Show MeSH

Related in: MedlinePlus

A,B: Thoracic CT of patient UPN 2106425: an 8-month-old female with respiratory distress at diagnosis. A: Huge enlargement of the thymus (black arrows). B: Evolution after a 6-week course of vinblastine and steroid; CT demonstrates decreased thymus involvement. C–F: Patient UPN 1506627, seen at 4 months of age for a skin rash. C: Chest X-rays with thymus enlargement. D: Chest X-rays after a 6-week course of vinblastine, showing normalization of thymus size. E: CT scan at time of initial diagnosis with thymus enlargement (*) and tracheal pushback (white arrow). F: Evolution after a 6-week course of vinblastine and steroid, demonstrating the decreased thymus size, but calcifications are now present (arrowhead). G,H: Thoracic CT of patient UPN 1506102. G: Presence of multiple calcifications (arrowheads). H: Coexistence of intra-thymic cysts (white arrows).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
getmorefigures.php?uid=PMC3824083&req=5

fig02: A,B: Thoracic CT of patient UPN 2106425: an 8-month-old female with respiratory distress at diagnosis. A: Huge enlargement of the thymus (black arrows). B: Evolution after a 6-week course of vinblastine and steroid; CT demonstrates decreased thymus involvement. C–F: Patient UPN 1506627, seen at 4 months of age for a skin rash. C: Chest X-rays with thymus enlargement. D: Chest X-rays after a 6-week course of vinblastine, showing normalization of thymus size. E: CT scan at time of initial diagnosis with thymus enlargement (*) and tracheal pushback (white arrow). F: Evolution after a 6-week course of vinblastine and steroid, demonstrating the decreased thymus size, but calcifications are now present (arrowhead). G,H: Thoracic CT of patient UPN 1506102. G: Presence of multiple calcifications (arrowheads). H: Coexistence of intra-thymic cysts (white arrows).

Mentions: At diagnosis, six patients had lymphopenia with deficiency of CD4 and CD8 T lymphocytes, which was profound in two cases among the 14 patients assessable for these criteria. Figure 1 depicts the lymphocyte status of patient UPN 1506627, both at diagnosis and during therapy; this patient's iconography is reported in Figure 2A,B. One child had hypogammaglobulinemia. In the other patients, the immunological control was normal or not checked.


Thymus and mediastinal node involvement in childhood Langerhans cell histiocytosis: long-term follow-up from the French national cohort.

Ducassou S, Seyrig F, Thomas C, Lambilliotte A, Marec-Berard P, Berger C, Plat G, Brugiere L, Ouache M, Barkaoui M, Armari-Alla C, Lutz P, Leverger G, Rialland X, Mansuy L, Pacquement H, Jeziorski E, Gandemer V, Chalard F, Chateil JF, Tazi A, Emile JF, Donadieu J, Investigators of the French LCH Study Gro - Pediatr Blood Cancer (2013)

A,B: Thoracic CT of patient UPN 2106425: an 8-month-old female with respiratory distress at diagnosis. A: Huge enlargement of the thymus (black arrows). B: Evolution after a 6-week course of vinblastine and steroid; CT demonstrates decreased thymus involvement. C–F: Patient UPN 1506627, seen at 4 months of age for a skin rash. C: Chest X-rays with thymus enlargement. D: Chest X-rays after a 6-week course of vinblastine, showing normalization of thymus size. E: CT scan at time of initial diagnosis with thymus enlargement (*) and tracheal pushback (white arrow). F: Evolution after a 6-week course of vinblastine and steroid, demonstrating the decreased thymus size, but calcifications are now present (arrowhead). G,H: Thoracic CT of patient UPN 1506102. G: Presence of multiple calcifications (arrowheads). H: Coexistence of intra-thymic cysts (white arrows).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3824083&req=5

fig02: A,B: Thoracic CT of patient UPN 2106425: an 8-month-old female with respiratory distress at diagnosis. A: Huge enlargement of the thymus (black arrows). B: Evolution after a 6-week course of vinblastine and steroid; CT demonstrates decreased thymus involvement. C–F: Patient UPN 1506627, seen at 4 months of age for a skin rash. C: Chest X-rays with thymus enlargement. D: Chest X-rays after a 6-week course of vinblastine, showing normalization of thymus size. E: CT scan at time of initial diagnosis with thymus enlargement (*) and tracheal pushback (white arrow). F: Evolution after a 6-week course of vinblastine and steroid, demonstrating the decreased thymus size, but calcifications are now present (arrowhead). G,H: Thoracic CT of patient UPN 1506102. G: Presence of multiple calcifications (arrowheads). H: Coexistence of intra-thymic cysts (white arrows).
Mentions: At diagnosis, six patients had lymphopenia with deficiency of CD4 and CD8 T lymphocytes, which was profound in two cases among the 14 patients assessable for these criteria. Figure 1 depicts the lymphocyte status of patient UPN 1506627, both at diagnosis and during therapy; this patient's iconography is reported in Figure 2A,B. One child had hypogammaglobulinemia. In the other patients, the immunological control was normal or not checked.

Bottom Line: Thirteen cases (35%) were diagnosed because of MI-related symptoms, including respiratory distress (N = 4), superior venous cava syndrome (N = 2), and/or cough and polypnea (N = 10).The overall 5-year survival was 87.1%, and immunodeficiency was not detected as a sequel.MI in LCH mainly occurs in young children, and diagnosis was based on CT showing thymus enlargement and calcifications.

View Article: PubMed Central - PubMed

Affiliation: Service D'hémato Oncologie Pédiatrique, CHU de Strasbourg, Strasbourg, France; Service D'hémato Oncologie Pédiatrique, CHU de Bordeaux, Bordeaux, France.

Show MeSH
Related in: MedlinePlus