Difference between revisions of "Immune thrombocytopenic purpura"

Immune thrombocytopenic purpura
	Diagnosis in short
Synonyms	immune thrombocytopenia
LM	germinal centres in white pulp (~55% of cases), neutrophils in the red pulp (~67% of cases), macrophages in the red pulp
Subtypes	clinical: primary, secondary
Gross	+/-prominent white nodules (spleen), normal appearance (most common)
Site	systemic - see spleen
Symptoms	bleeding
Prevalence	uncommon
Blood work	thrombocytopenia, antiplatelet antibodies
Prognosis	dependent on underlying cause
Treatment	corticosteroids, possibly splenectomy

Revision as of 19:02, 1 September 2016

Immune thrombocytopenic purpura, abbreviated ITP, is a rare condition.

General

Presentation:

Bleeding - usually.^[1]

Blood work:

Thrombocytopenia.
Antiplatelet antibodies.

Treatment:

Usually treated with corticosteroids.^[1]
- Possibly retuximab.^[2]
Refractory cases get a splenectomy.

Prevalence:

Uncommon.

Classification

Primary - diagnosis of exclusion.
Secondary.
- Autoimmune.
  - Systemic lupus erythematosus.^[3]
- Viral.
- Bacterial - possibly Helicobacter pylori.^[4]

Gross

Features:^[5]

Typically ~ 100 grams.
+/-Prominent white nodules (AKA Malpighian corpuscles) - uncommon.

Microscopic

Features:^[5]

Germinal centres in white pulp (~55% of cases)
- Classically described as "proliferative".
Neutrophils in the red pulp (~67% of cases).
Macrophages in the red pulp (~25% of cases). †

Notes:

Changes not dependent on titre of antiplatelet antibodies.^[5]
† May be a finding associated with treatment.^[5]
The classic changes are proliferation of lymphoid germinal centres and red pulp PMNs.

Images

Sign out

Spleen, Splenetectomy:
     - Spleen with prominent red pulp macrophages and expanded germinal 
       centres, pending hematopathology consult.

Block letters

SPLEEN, SPLENECTOMY:
- SPLEEN WITH CHANGES COMPATIBLE WITH IMMUNE THROMBOCYTOPENIC PURPURA.

Micro

The sections show spleen with increased numbers of red pulp macrophages and siderophages. Rare scattered red pulp neutrophils are seen. Rare plasma cells are seen in the red pulp. Lymphoid follicles do not show significant expansion.

References

↑ ^1.0 ^1.1 Kistangari G, McCrae KR (June 2013). "Immune thrombocytopenia". Hematol. Oncol. Clin. North Am. 27 (3): 495–520. doi:10.1016/j.hoc.2013.03.001. PMID 23714309.
↑ Zhang, C.; Liu, HF.; Chen, XH.; Gao, L.; Gao, L.; Liu, Y.; Kong, PY.; Sun, AH. et al. (Mar 2014). "Is splenectomy necessary for immune thrombocytopenic purpura? The role of rituximab in patients with corticosteroid resistance in a single-center experience.". Clin Ther 36 (3): 385-8. doi:10.1016/j.clinthera.2014.01.017. PMID 24594069.
↑ Cines DB, Liebman H, Stasi R (January 2009). "Pathobiology of secondary immune thrombocytopenia". Semin. Hematol. 46 (1 Suppl 2): S2–14. doi:10.1053/j.seminhematol.2008.12.005. PMC 2682438. PMID 19245930. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2682438/.
↑ Kuwana M (January 2014). "Helicobacter pylori-associated immune thrombocytopenia: clinical features and pathogenic mechanisms". World J. Gastroenterol. 20 (3): 714–23. doi:10.3748/wjg.v20.i3.714. PMC 3921481. PMID 24574745. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3921481/.
↑ ^5.0 ^5.1 ^5.2 ^5.3 Hayes MM, Jacobs P, Wood L, Dent DM (September 1985). "Splenic pathology in immune thrombocytopenia". J. Clin. Pathol. 38 (9): 985–8. PMC 499346. PMID 4044880. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC499346/.

[pmid23714309-1] 1.0 ^1.1 Kistangari G, McCrae KR (June 2013). "Immune thrombocytopenia". Hematol. Oncol. Clin. North Am. 27 (3): 495–520. doi:10.1016/j.hoc.2013.03.001. PMID 23714309.

[2] Zhang, C.; Liu, HF.; Chen, XH.; Gao, L.; Gao, L.; Liu, Y.; Kong, PY.; Sun, AH. et al. (Mar 2014). "Is splenectomy necessary for immune thrombocytopenic purpura? The role of rituximab in patients with corticosteroid resistance in a single-center experience.". Clin Ther 36 (3): 385-8. doi:10.1016/j.clinthera.2014.01.017. PMID 24594069.

[3] Cines DB, Liebman H, Stasi R (January 2009). "Pathobiology of secondary immune thrombocytopenia". Semin. Hematol. 46 (1 Suppl 2): S2–14. doi:10.1053/j.seminhematol.2008.12.005. PMC 2682438. PMID 19245930. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2682438/.

[4] Kuwana M (January 2014). "Helicobacter pylori-associated immune thrombocytopenia: clinical features and pathogenic mechanisms". World J. Gastroenterol. 20 (3): 714–23. doi:10.3748/wjg.v20.i3.714. PMC 3921481. PMID 24574745. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3921481/.

[pmid4044880-5] 5.0 ^5.1 ^5.2 ^5.3 Hayes MM, Jacobs P, Wood L, Dent DM (September 1985). "Splenic pathology in immune thrombocytopenia". J. Clin. Pathol. 38 (9): 985–8. PMC 499346. PMID 4044880. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC499346/.

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