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Stains & CD markers
CD103

Moiz Vora, M.D.
Maurice Richardson, M.D.

Last author update: 22 June 2023
Last staff update: 22 June 2023

Copyright: 2022-2024, PathologyOutlines.com, Inc.

PubMed Search: CD103

Moiz Vora, M.D.
Maurice Richardson, M.D.
Table of Contents
Definition / general | Essential features | Terminology | Pathophysiology | Diagrams / tables | Clinical features | Interpretation | Uses by pathologists | Prognostic factors | Microscopic (histologic) description | Microscopic (histologic) images | Positive staining - normal | Positive staining - disease | Negative staining | Sample pathology report | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2
Cite this page: Vora M, Richardson M. CD103. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/stainsCD103.html. Accessed December 24th, 2024.
Definition / general
  • CD103 is a heterodimeric transmembrane surface receptor that is expressed by several cell types of the immune system and is involved in cell to cell or cell to matrix interactions
  • CD103 mediates cell adhesion, migration and lymphocyte homing through interaction with E-cadherin, which is expressed in epithelial cells
Essential features
Terminology
  • Integrin alpha Eβ7 (ITGAE)
  • Integrin alpha E (αE)
    • αE subunit first described as human mucosal lymphocyte 1 (HML1) antigen
Pathophysiology
  • CD103 is a heterodimer complex; the integrin αE chain (CD103 in the strict sense) dimerizes exclusively with the β7 integrin subunit
  • CD103 is the first integrin discovered that binds to a cadherin, namely E-cadherin
  • Binding of CD103 is heterophilic
    • αE subunit binds through its metal ion dependent coordination site (MIDAS) motif to the tip of the BC loop of E-cadherin (J Exp Med 2000;191:1555)
    • β7 chain does not bind directly to E-cadherin but appears to have more of a regulatory function in this process
  • CD103 mediates lymphocyte retention in epithelial tissues and allows immunocytes to maintain close contact with both peripheral lymphoid and nonlymphoid tissue (through binding to E-cadherin) (Nature 1994;372:190)
  • Transforming growth factor β (TGFβ) has been found to consistently upregulate its expression
    • This cytokine induces CD103 on human cytotoxic lymphocytes in conjunction with T cell receptor (TCR) activation
Diagrams / tables

Images hosted on other servers:

Domain structure of the αE(CD103)β7 integrin

Clinical features
Interpretation
  • Membranous (primarily) and cytoplasmic (variable intensity) with overlay of irregular cytoplasmic borders (consistent with projections) (Am J Clin Pathol 2013;139:220)
Uses by pathologists
Prognostic factors
Microscopic (histologic) description
  • CD103 expression is uniform with high intensity in cases of bone marrow involvement by hairy cell leukemia (in a diffuse interstitial pattern, ≥ 50% involvement)
  • CD103 expression follows the distribution of neoplastic cells in cases of a patchy intrasinusoidal / interstitial pattern of bone marrow involvement (low level, 15% involvement)
  • Reference: Am J Clin Pathol 2013;139:220
Microscopic (histologic) images

Contributed by Maurice Richardson, M.D. and Moiz Vora, M.D.

Diffuse lymphoid infiltrate

CD103+ neoplastic lymphocytes

Intrasinusoidal lymphoid infiltrate

CD103+ neoplastic lymphocytes

Positive staining - normal
Positive staining - disease
Negative staining
Sample pathology report
  • Bone marrow, posterior iliac crest, aspirate and core biopsy:
    • Hypercellular marrow with involvement by CD103+ mature B cell neoplasm (see comment)
    • Comment: The trephine core biopsy shows intravascular and patchy interstitial infiltration by small to intermediate sized lymphoid cells with asymmetric cytoplasmic projections and occasional nucleoli. These cells are positive for CD20, bright CD11c, bright CD22, CD103 and show kappa light chain restriction. These cells are negative for CD5, CD10, CD25 and CD38.
    • The overall morphologic and immunophenotypic features are consistent with involvement by a splenic origin lymphoma. Absence of CD25 coexpression argues against a diagnosis of classical hairy cell leukemia. The primary diagnostic considerations include hairy cell leukemia variant and splenic marginal zone lymphoma. An additional differential may include splenic diffuse red pulp lymphoma (SDRPL). The morphologic features reflect a low grade process. There is no overt large cell transformation observed.
    • Correlation with clinical findings and additional pertinent laboratory and radiographic studies is recommended for comprehensive assessment. This case (core biopsy) was submitted for internal quality assurance review.
Board review style question #1


A 59 year old man visits his primary care doctor to be evaluated for progressive weakness and decreased exercise tolerance. Physical examination shows splenomegaly and petechial bruises on the upper limbs bilaterally. Laboratory studies reveal pancytopenia. Peripheral smear reveals the findings in the image above and flow cytometry shows a mature monotypic B cell population coexpressing bright CD20, CD22, CD25, CD11c, CD103, FMC7, CD200 and lambda light chain restriction. Which of the following is the most likely diagnosis?

  1. Adult T cell leukemia / lymphoma (ATLL)
  2. Angioimmunoblastic T cell lymphoma (AITL)
  3. Hairy cell leukemia (HCL)
  4. Splenic diffuse red pulp small B cell lymphoma (SDRPL)
  5. T cell large granular lymphocytic leukemia (TLGL)
Board review style answer #1
C. Hairy cell leukemia (HCL). The immunoprofile (particularly coexpression of CD11c, CD25 and CD103) and cytologic features (intermediate sized lymphocytes with circumferential cytoplasmic projections) are most consistent with hairy cell leukemia. The alternate disease entities listed either do not (or seldomly) coexpress CD103 with the other markers listed above (i.e., AITL, TLGL, SDRPL) or have different cytologic features in the peripheral blood (i.e., ATLL shows multilobed nuclei flower cells in the peripheral blood).

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Reference: CD103
Board review style question #2
A 40 year old man presents to his physician with fever and left upper quadrant pain. Subsequent clinical evaluation shows anemia and monocytopenia. A bone marrow biopsy evaluation reveals a B cell lymphoproliferative disorder with the following immunoprofile: CD20+, CD25+, CD103+, annexin A1+, DBA44+. A diagnosis of hairy cell leukemia is made. Which of the following is the characteristic staining pattern of CD103 in this disease entity?

  1. Cytoplasmic
  2. Membranous (primarily) and cytoplasmic
  3. Nuclear
  4. Perinuclear dot-like
  5. Perinuclear golgi
Board review style answer #2
B. Membranous (primarily) and cytoplasmic. The most characteristic immunohistochemical pattern of CD103 staining is described in the literature as membranous (primarily) and cytoplasmic (variable intensity) with overlay of irregular cytoplasmic borders (consistent with projections). Since CD103 is a transmembrane surface receptor, the CD103 antibody would bind primarily to the cell membrane; therefore, the alternate choices (nuclear, perinuclear golgi or dot-like and cytoplasmic) are incorrect.

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Reference: CD103
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