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Autoimmune Lymphoproliferative Syndrome: A Syndrome Associated with Inherited Genetic Defects That Impair Lymphocytic Apoptosis—CT and US Features¹

¹ From the Diagnostic Radiology Department, Warren Grant Magnuson Clinical Center, National Institutes of Health, Bldg 10, Rm 1C660, 10 Center Dr, MSC 1182, Bethesda, MD 20892-1182 (N.A.A., A.J.D.); the Laboratories of Clinical Investigation (J.K.D., U.A.L., S.E.S.) and Immunoregulation (M.C.S), National Institute of Allergy and Infectious Diseases, Bethesda, Md; the Laboratory of Pathology, National Cancer Institute, Bethesda, Md (E.S.J.); and the National Human Genome Research Institute, Bethesda, Md (J.M.P.). Received July 6, 1998; revision requested August 27; final revision received October 26; accepted January 19, 1999. Address reprint requests to N.A.A. (e-mail: navila@nih.gov).

View larger version (113K): [in a new window]   Figure 1a. CT images of the neck in a 4-year-old patient with ALPS. (a) Axial section at the level of the mandible shows extensive cervical adenopathy (arrow). (b) Axial section at the base of the neck shows extensive supraclavicular adenopathy (large arrow) and submental adenopathy (small arrow).

View larger version (110K): [in a new window]   Figure 1b. CT images of the neck in a 4-year-old patient with ALPS. (a) Axial section at the level of the mandible shows extensive cervical adenopathy (arrow). (b) Axial section at the base of the neck shows extensive supraclavicular adenopathy (large arrow) and submental adenopathy (small arrow).

View larger version (146K): [in a new window]   Figure 2. Transverse gray-scale US image of the neck in a 16-year-old patient with ALPS shows an enlarged lymph node (arrow) with echogenicity similar to that of the thyroid gland. CCA = common carotid artery, JV = jugular vein, LN = lymph node, THY = thyroid.

View larger version (64K): [in a new window]   Figure 3. Transverse color Doppler US image of the submental region in a 10-year-old patient with ALPS shows moderate vascularity (arrows) within the submental lymph nodes.

View larger version (221K): [in a new window]   Figure 4a. Histologic results from a cervical lymph node biopsy in a patient with ALPS shows florid reactive hyperplasia and a markedly expanded CD3+CD4-CD8- subset of T cells. (a) Photomicrograph obtained with hematoxylin-eosin staining shows preservation of the lymph node architecture (curved arrow), marked reactive follicular hyperplasia, and paracortical expansion (straight arrow), with immunoblasts and plasma cells. (b–d) Photomicrographs obtained following staining of lymph node sections for (b) CD3, (c) CD4, and (d) CD8 show extensive accumulation of T cells (arrow) in the paracortical region. The absence of staining for (c) CD4 or (d) CD8 in the lymph node photomicrographs shows that most CD3+ cells are CD4- and CD8-. (In a–d, original magnification, x100.)

View larger version (204K): [in a new window]   Figure 4b. Histologic results from a cervical lymph node biopsy in a patient with ALPS shows florid reactive hyperplasia and a markedly expanded CD3+CD4-CD8- subset of T cells. (a) Photomicrograph obtained with hematoxylin-eosin staining shows preservation of the lymph node architecture (curved arrow), marked reactive follicular hyperplasia, and paracortical expansion (straight arrow), with immunoblasts and plasma cells. (b–d) Photomicrographs obtained following staining of lymph node sections for (b) CD3, (c) CD4, and (d) CD8 show extensive accumulation of T cells (arrow) in the paracortical region. The absence of staining for (c) CD4 or (d) CD8 in the lymph node photomicrographs shows that most CD3+ cells are CD4- and CD8-. (In a–d, original magnification, x100.)

View larger version (220K): [in a new window]   Figure 4c. Histologic results from a cervical lymph node biopsy in a patient with ALPS shows florid reactive hyperplasia and a markedly expanded CD3+CD4-CD8- subset of T cells. (a) Photomicrograph obtained with hematoxylin-eosin staining shows preservation of the lymph node architecture (curved arrow), marked reactive follicular hyperplasia, and paracortical expansion (straight arrow), with immunoblasts and plasma cells. (b–d) Photomicrographs obtained following staining of lymph node sections for (b) CD3, (c) CD4, and (d) CD8 show extensive accumulation of T cells (arrow) in the paracortical region. The absence of staining for (c) CD4 or (d) CD8 in the lymph node photomicrographs shows that most CD3+ cells are CD4- and CD8-. (In a–d, original magnification, x100.)

View larger version (219K): [in a new window]   Figure 4d. Histologic results from a cervical lymph node biopsy in a patient with ALPS shows florid reactive hyperplasia and a markedly expanded CD3+CD4-CD8- subset of T cells. (a) Photomicrograph obtained with hematoxylin-eosin staining shows preservation of the lymph node architecture (curved arrow), marked reactive follicular hyperplasia, and paracortical expansion (straight arrow), with immunoblasts and plasma cells. (b–d) Photomicrographs obtained following staining of lymph node sections for (b) CD3, (c) CD4, and (d) CD8 show extensive accumulation of T cells (arrow) in the paracortical region. The absence of staining for (c) CD4 or (d) CD8 in the lymph node photomicrographs shows that most CD3+ cells are CD4- and CD8-. (In a–d, original magnification, x100.)

View larger version (62K): [in a new window]   Figure 5a. Axial CT sections of the chest and abdomen in an 11-year-old patient with ALPS show enhancing axillary, retroperitoneal, and inguinal lymph nodes. (a) Section near the origin of the great vessels from the aortic arch demonstrates enhancement of axillary adenopathy (arrowheads). Also note mediastinal adenopathy (arrow) in the pretracheal space, which separates the right brachiocephalic vein and the brachiocephalic artery. (b) Precontrast study below the left renal vein shows retroperitoneal lymph nodes (arrow). (c) Postcontrast study below the left renal vein demonstrates mild enhancement of the retroperitoneal nodes (arrow). (d) Section at the level of the acetabuli shows enhancement of the inguinal nodes (arrows).

View larger version (139K): [in a new window]   Figure 5b. Axial CT sections of the chest and abdomen in an 11-year-old patient with ALPS show enhancing axillary, retroperitoneal, and inguinal lymph nodes. (a) Section near the origin of the great vessels from the aortic arch demonstrates enhancement of axillary adenopathy (arrowheads). Also note mediastinal adenopathy (arrow) in the pretracheal space, which separates the right brachiocephalic vein and the brachiocephalic artery. (b) Precontrast study below the left renal vein shows retroperitoneal lymph nodes (arrow). (c) Postcontrast study below the left renal vein demonstrates mild enhancement of the retroperitoneal nodes (arrow). (d) Section at the level of the acetabuli shows enhancement of the inguinal nodes (arrows).

View larger version (133K): [in a new window]   Figure 5c. Axial CT sections of the chest and abdomen in an 11-year-old patient with ALPS show enhancing axillary, retroperitoneal, and inguinal lymph nodes. (a) Section near the origin of the great vessels from the aortic arch demonstrates enhancement of axillary adenopathy (arrowheads). Also note mediastinal adenopathy (arrow) in the pretracheal space, which separates the right brachiocephalic vein and the brachiocephalic artery. (b) Precontrast study below the left renal vein shows retroperitoneal lymph nodes (arrow). (c) Postcontrast study below the left renal vein demonstrates mild enhancement of the retroperitoneal nodes (arrow). (d) Section at the level of the acetabuli shows enhancement of the inguinal nodes (arrows).

View larger version (128K): [in a new window]   Figure 5d. Axial CT sections of the chest and abdomen in an 11-year-old patient with ALPS show enhancing axillary, retroperitoneal, and inguinal lymph nodes. (a) Section near the origin of the great vessels from the aortic arch demonstrates enhancement of axillary adenopathy (arrowheads). Also note mediastinal adenopathy (arrow) in the pretracheal space, which separates the right brachiocephalic vein and the brachiocephalic artery. (b) Precontrast study below the left renal vein shows retroperitoneal lymph nodes (arrow). (c) Postcontrast study below the left renal vein demonstrates mild enhancement of the retroperitoneal nodes (arrow). (d) Section at the level of the acetabuli shows enhancement of the inguinal nodes (arrows).

View larger version (148K): [in a new window]   Figure 6. Longitudinal US image of periportal adenopathy in a 9-year-old patient with ALPS shows lymph nodes that are isoechoic (short arrow) and hyperechoic (long arrow) relative to the liver. (Crosshairs indicate location of calipers.)

View larger version (88K): [in a new window]   Figure 7. Axial CT image at the level of the anterior mediastinum in a 4-year-old patient with ALPS demonstrates a diffusely enlarged thymus (arrow).

View larger version (103K): [in a new window]   Figure 8. Axial CT image at the level of the anterior mediastinum in a 9-year-old patient with ALPS demonstrates an enlarged thymus (arrow) with a multinodular appearance.