Spleen

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The spleen is a forgotten organ.

Normal histology

Splenic enlargement

DDx by compartment

Red pulp benign

Red pulp malignant

Specific disorders

Hyaloserositis

  • Properly referred to as hyaloserositis of the spleen.
  • Capsule of the spleen is white - resembles sugar-coating; also known as sugar-coated spleen.[6]
  • Importance: none - benign.

Image(s):

Mononucleosis

General

  • EBV infection.
  • +/-Massive splenic enlargement.

Clinical:

  • Monospot test +ve.

Microscopic

Features:

  • Atypical lymphoid cells.
    • Abundant basophilic cytoplasm.
    • Cells indented by adjacent RBCs on blood smears.[7]

Image: Mononucleosis (med.utah.edu).[7]

Littoral cell angioma

For angioma see vascular malformations.
  • AKA littoral cell angioma of the spleen.

General

Features:[8]

  • Rare.
  • Benign.

Clinical:[8][9]

  • Pyrexia (fever), splenomegaly, weight loss, solitary hypodense lesion on imaging.

Note:

  • Littoral cells = line vascular channels of the red pulp.[8]

Microscopic

Features:[8]

  • Anastoming, small vascular channels.
  • Cystic spaces.
    • May have papillary projections.[9]

DDx:[8]

Images:

IHC

Features:[8]

  • CD68 +ve.
  • CD31 +ve

Splenic hamartoma

General

  • Benign.
  • Women > men.[12]

Microscopic

Features:[13]

  • Variable size irregular vascular channels
  • Red pulp-like disorganized stroma
  • +/-Lymphoid follicles.

IHC

Features:[13]

  • CD8 +ve endothelial cells.

Weird stuff

  • Dendritic cell tumours.[4]
    • Interdigitating dendritic cell tumour.
    • Follicular dendritic cell tumour.

Follicular dendritic cell tumour

  • AKA follicular dendritic cell sarcoma,[14] dendritic reticulum cell sarcoma, (???) follicular dendritic cell neoplasm. (???)

General

Microscopic

Features:[15]

  • Oval or spindle-shaped cellular & nuclear morphology.
  • Variable architecture (sheets, fascicles, whorles, storiform pattern).
  • Nuclei:
    • Small nucleoli.
    • Clear or dispersed chromatin.
  • Multinucleated cells.
  • Interspersed small lymphocytes - distinctive feature.
  • +/-Necrosis.
  • +/-Marked nuclear atypia.
  • +/-Abundant mitoses.

Images:

IHC

Features:[15]

  • CD21 +ve.
  • CD35 +ve.
  • Ki-M4p +ve
  • Ki-FDRC1p +ve.
  • Vimentin +ve.
  • S-100 +ve/-ve.
  • Muscle-specific actin +ve/-ve.
  • EMA +ve/-ve.

Hepatosplenic T-cell lymphoma

  • Abbreviated HSTL.

General

  • Rare.
  • Prognosis: poor.

Subtypes:[16]

  1. Hepatosplenic γδ T-cell lymphoma.
    • May be written Hepatosplenic gamma/delta T-cell lymphoma.
  2. Hepatosplenic αβ T-cell lymphoma.
    • May be written Hepatosplenic alpha/beta T-cell lymphoma.

Clinical triad:

  1. Hepatosplenomegaly.
  2. Cytopenias (anemia, thrombocytopenia).
  3. Sinusoidal tropism.

Microscopic

Features:[5][16]

  • Small cell lymphoma/Intermediate cell lymphoma.
    • +/-"Folded" nuclei.

DDx:

Images:

IHC

Features:[5]

  • CD4 -ve.
  • CD8 -ve.
  • NK cell-associated antigens +ve:[17]
    • CD56,[18] CD11c, CD16.
  • Effector proteins +ve.[17]
    • Perforin, granzyme B, TIA-1, Fas ligand.

See also

References

  1. Bennett, M.; Schechter, GP. (Apr 2010). "Treatment of splenic marginal zone lymphoma: splenectomy versus rituximab.". Semin Hematol 47 (2): 143-7. doi:10.1053/j.seminhematol.2010.01.004. PMID 20350661.
  2. Baseggio L, Traverse-Glehen A, Callet-Bauchu E, et al. (March 2011). "Relevance of a scoring system including CD11c expression in the identification of splenic diffuse red pulp small B-cell lymphoma (SRPL)". Hematol Oncol 29 (1): 47–51. doi:10.1002/hon.957. PMID 20677173.
  3. Maakaroun, NR.; Moanna, A.; Jacob, JT.; Albrecht, H. (Mar 2010). "Viral infections associated with haemophagocytic syndrome.". Rev Med Virol 20 (2): 93-105. doi:10.1002/rmv.638. PMID 20127750.
  4. 4.0 4.1 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 596. ISBN 978-0781765275.
  5. 5.0 5.1 5.2 Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 595. ISBN 978-0781765275.
  6. URL: http://www.drugs.com/dict/sugar-coated-spleen.html. Accessed on: 1 September 2010.
  7. 7.0 7.1 URL: http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html. Accessed on: 4 December 2011.
  8. 8.0 8.1 8.2 8.3 8.4 8.5 Tan, YM.; Chuah, KL.; Wong, WK. (Jul 2004). "Littoral cell angioma of the spleen.". Ann Acad Med Singapore 33 (4): 524-6. PMID 15329769. http://www.annals.edu.sg/pdf200408/V33N4p524.pdf.
  9. 9.0 9.1 Dascalescu, CM.; Wendum, D.; Gorin, NC. (Sep 2001). "Littoral-cell angioma as a cause of splenomegaly.". N Engl J Med 345 (10): 772-3. doi:10.1056/NEJM200109063451016. PMID 11547761. http://www.nejm.org/doi/full/10.1056/NEJM200109063451016.
  10. URL: http://www.merriam-webster.com/dictionary/littoral. Accessed on: 24 July 2011.
  11. URL: http://dictionary.reference.com/browse/littoral. Accessed on: 24 July 2011.
  12. Sankar, S.; Thanka, J.; Jagdishchandrabose, S.; Rajendran, S.. "Splenic hamartoma: a rare vascular space occupying lesion of the spleen.". Indian J Pathol Microbiol 54 (1): 223-5. doi:10.4103/0377-4929.77416. PMID 21393931.
  13. 13.0 13.1 Lee, H.; Maeda, K. (Jan 2009). "Hamartoma of the spleen.". Arch Pathol Lab Med 133 (1): 147-51. doi:10.1043/1543-2165-133.1.147. PMID 19123729.
  14. 14.0 14.1 Leipsic, JA.; McAdams, HP.; Sporn, TA. (Jun 2007). "Follicular dendritic cell sarcoma of the mediastinum.". AJR Am J Roentgenol 188 (6): W554-6. doi:10.2214/AJR.04.1530. PMID 17515347.
  15. 15.0 15.1 15.2 15.3 Perez-Ordoñez, B.; Rosai, J. (May 1998). "Follicular dendritic cell tumor: review of the entity.". Semin Diagn Pathol 15 (2): 144-54. PMID 9606805.
  16. 16.0 16.1 URL: http://www.webpathology.com/image.asp?case=378&n=15. Accessed on: 22 December 2011.
  17. 17.0 17.1 Salhany, KE.; Feldman, M.; Kahn, MJ.; Peritt, D.; Schretzenmair, RD.; Wilson, DM.; DiPaola, RS.; Glick, AD. et al. (Jun 1997). "Hepatosplenic gammadelta T-cell lymphoma: ultrastructural, immunophenotypic, and functional evidence for cytotoxic T lymphocyte differentiation.". Hum Pathol 28 (6): 674-85. PMID 9191001.
  18. Niitsu, N.; Kohri, M.; Togano, T.; Nakamine, H.; Nakamura, S.; Iwabuchi, K.; Higashihara, M. (Nov 2004). "Development of hepatosplenic gammadelta T-cell lymphoma with pancytopenia during early pregnancy: a case report and review of the literature.". Eur J Haematol 73 (5): 367-71. doi:10.1111/j.1600-0609.2004.00300.x. PMID 15458516.

External links