Spleen

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The spleen is a forgotten organ.

Normal histology

  • Capsule.
  • Red pulp - red blood cells.
  • White pulp - white blood cells.
  • Marginal zone - between red pulp and white pulp.[1]

Images

  • Normal spleen (left) and splenic hamartoma (right). H&E stain. (WC)

Gross pathology

Terminology Etiology Description Other
Ruptured spleen trauma hemorrhagic, capsule disrupted possible assocations mononucleosis, medical procedure - esp. colonoscopy,[2] others
"Lardaceous spleen"[3] amyloidosis waxy, pale, grey also see sago spleen
"Sago spleen" amyloidosis nodular, tapioca-like appearance also see lardaceous spleen
Splenic infarct vascular occulsion wedge shaped -- periphery -

Images:

Splenic enlargement

Lymphoid neoplasms of the spleen

Lymphomas of the spleen in order of prevalence - in a series of 115 cases:[5]

  1. Diffuse large B-cell lymphoma (DLBCL).
  2. Splenic marginal zone lymphoma (SMZL).[6]
  3. Follicular lymphoma.
  4. Splenic B-cell lymphoma, unclassifiable.
  5. Peripheral T-cell lymphoma, not otherwise specified.

Less common lymphoid neoplasms of the spleen:

DDx by compartment

White pulp malignant

Red pulp benign

Red pulp malignant

Specific disorders

Splenic laceration

Main article: Splenic laceration

Hyaloserositis of the spleen

  • AKA sugar-coated spleen.

General

Gross

  • Capsule of the spleen is white - resembles sugar-coating.[10]

Microscopic

Features:

  • Hyaline material adherent to splenic capsule.
    • Hyaline material = pink acellular crap on a H&E stain.

Images:

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.[11]

Images:

Flow cytometry

Littoral cell angioma

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

General

Features:[13]

  • Rare.
  • Benign.

Clinical:[13][14]

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

Note:

  • Littoral cells = line vascular channels of the red pulp.[13]

Microscopic

Features:[13]

  • Anastoming, small vascular channels.
  • Cystic spaces.
    • May have papillary projections.[14]

DDx:[13]

Images:

IHC

Features:[13]

  • CD68 +ve.
  • CD31 +ve

Others:

  • CD34 -ve.
  • Factor VIII +ve.

Splenic hamartoma

General

  • Benign.
  • Women > men.[17]

Gross

  • Red lesion.
  • Well-circumscribed.

Image:

Microscopic

Features:[18]

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

Images:

IHC

Features:[18]

  • CD8 +ve endothelial cells.

Splenic infarct

General

Classic textbook causes:[19]

Usual causes:[19]

  • Hematologic malignancy.
  • Intracardiac thrombus.
  • Bacterial endocarditis.

Clinical:[19]

  • Left upper quadrant pain ~ 1/3 of cases.
  • Fever ~ 1/3 of cases.
  • Leukocytosis ~ 1/2 of cases.

Gross

  • Classically wedge-shaped; triangular on section.
    • The base of the triangle runs along the surface.
    • The apex points to the obstructed vessel that lead to the infarct.

Microscopic

See necrosis.

Weird stuff

  • Dendritic cell tumours.[8]
    • Interdigitating dendritic cell tumour.
    • Follicular dendritic cell tumour.

Follicular dendritic cell tumour

  • Abbreviated FDCT.
  • AKA follicular dendritic cell sarcoma (abbreviated FDCS),[20] dendritic reticulum cell sarcoma, (???) follicular dendritic cell neoplasm. (???)

General

Microscopic

Features:[21]

  • 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.

DDx:

Images:

IHC

Features:[21]

  • 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:[23]

  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:[9][23]

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

DDx:

Images:

IHC

Features:[9]

  • CD4 -ve.
  • CD8 -ve.
  • NK cell-associated antigens +ve:[24]
    • CD56,[25] CD11c, CD16.
  • Effector proteins +ve.[24]
    • Perforin, granzyme B, TIA-1, Fas ligand.

Myelolipoma of the spleen

Main article: Myelolipoma

See also

References

  1. ↑ Humphrey, Peter A; Dehner, Louis P; Pfeifer, John D (2008). The Washington Manual of Surgical Pathology (1st ed.). Lippincott Williams & Wilkins. pp. 589. ISBN 978-0781765275.
  2. ↑ Aubrey-Bassler, FK.; Sowers, N. (Aug 2012). "613 cases of splenic rupture without risk factors or previously diagnosed disease: a systematic review.". BMC Emerg Med 12 (1): 11. doi:10.1186/1471-227X-12-11. PMID 22889306.
  3. ↑ Klatt, Edward C. (2006). Robbins and Cotran Atlas of Pathology (1st ed.). Saunders. pp. 91. ISBN 978-1416002741.
  4. ↑ 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.
  5. ↑ Shimizu-Kohno, K.; Kimura, Y.; Kiyasu, J.; Miyoshi, H.; Yoshida, M.; Ichikawa, R.; Niino, D.; Ohshima, K. (Sep 2012). "Malignant lymphoma of the spleen in Japan: a clinicopathological analysis of 115 cases.". Pathol Int 62 (9): 577-82. doi:10.1111/j.1440-1827.2012.02844.x. PMID 22924843.
  6. ↑ 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.
  7. ↑ 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.
  8. ↑ 8.0 8.1 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.
  9. ↑ 9.0 9.1 9.2 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.
  10. ↑ URL: http://www.drugs.com/dict/sugar-coated-spleen.html. Accessed on: 1 September 2010.
  11. ↑ 11.0 11.1 URL: http://library.med.utah.edu/WebPath/EXAM/IMGQUIZ/hpfrm.html. Accessed on: 4 December 2011.
  12. ↑ URL: http://path.upmc.edu/cases/case37/gross.html. Accessed on: 2 January 2012.
  13. ↑ 13.0 13.1 13.2 13.3 13.4 13.5 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.
  14. ↑ 14.0 14.1 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.
  15. ↑ URL: http://www.merriam-webster.com/dictionary/littoral. Accessed on: 24 July 2011.
  16. ↑ URL: http://dictionary.reference.com/browse/littoral. Accessed on: 24 July 2011.
  17. ↑ 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.
  18. ↑ 18.0 18.1 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.
  19. ↑ 19.0 19.1 19.2 Lawrence, YR.; Pokroy, R.; Berlowitz, D.; Aharoni, D.; Hain, D.; Breuer, GS.; Osler, W. (Jun 2010). "Splenic infarction: an update on William Osler's observations.". Isr Med Assoc J 12 (6): 362-5. PMID 20928991.
  20. ↑ 20.0 20.1 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.
  21. ↑ 21.0 21.1 21.2 21.3 Perez-Ordoñez, B.; Rosai, J. (May 1998). "Follicular dendritic cell tumor: review of the entity.". Semin Diagn Pathol 15 (2): 144-54. PMID 9606805.
  22. ↑ Alexiev, BA.; Sailey, CJ.; McClure, SA.; Ord, RA.; Zhao, XF.; Papadimitriou, JC. (2007). "Primary histiocytic sarcoma arising in the head and neck with predominant spindle cell component.". Diagn Pathol 2: 7. doi:10.1186/1746-1596-2-7. PMID 17324277. http://www.diagnosticpathology.org/content/2/1/7.
  23. ↑ 23.0 23.1 URL: http://www.webpathology.com/image.asp?case=378&n=15. Accessed on: 22 December 2011.
  24. ↑ 24.0 24.1 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.
  25. ↑ 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

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