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

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Micro

Sections show unremarkable splenic parenchyma. The nodules of white pulp are well-spaced and have germinal center formation. The red pulp has a normal vascularity and does not appear to be expanded. No nuclear atypia is apparent.

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

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.

Splenic hamartoma

Splenic infarct

General

Classic textbook causes:[13]

Usual causes:[13]

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

Clinical:[13]

  • 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),[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.

DDx:

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:[17]

  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][17]

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

DDx:

Images:

IHC

Features:[9]

  • CD4 -ve.
  • CD8 -ve.
  • NK cell-associated antigens +ve:[18]
    • CD56,[19] CD11c, CD16.
  • Effector proteins +ve.[18]
    • Perforin, granzyme B, TIA-1, Fas ligand.

Myelolipoma of the spleen

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 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.
  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. 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.
  17. 17.0 17.1 URL: http://www.webpathology.com/image.asp?case=378&n=15. Accessed on: 22 December 2011.
  18. 18.0 18.1 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.
  19. 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.

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