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{{Autoimmune lymphoproliferative syndrome}}
{{Autoimmune lymphoproliferative syndrome}}


'''Editor-In-Chief:''' David Teachey, MD [mailto:TEACHEYD@email.chop.edu]
'''Editor-In-Chief: {{CMG}}''' David Teachey, MD [mailto:TEACHEYD@email.chop.edu] {{AE}}{{SharmiB}}


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==Overview==


==Overview==
Common [[physical examination]] findings of [[Autoimmune lymphoproliferative syndrome]]([[ALPS]]) include [[lymphadenopathy]], [[Hepatomegaly]], or [[splenomegaly]]. The majority of [[patients]](80%) have enlarged, palpable, non -tender [[lymph nodes]] for an extended period of time. [[Cervical]], [[axillary]], [[inguinal]] [[lymphadenopathy]] are mostly found. But preauricular, submental, epitrochlear, mediastinal, and retroperitoneal nodes are detected occasionally. Moderate to massive [[splenomegaly]] is evident in 85% of [[patients]] with [[ALPS]]. Minor [[hepatomegaly]] is also a common finding. [[Lymphadenopathy]], [[splenomegaly]], [[hepatomegaly]] improve with age.
Patients with [disease name] usually appear [general appearance]. Physical examination of patients with [disease name] is usually remarkable for [finding 1], [finding 2], and [finding 3].


OR
==Physical Examination==
===Skin===


Common physical examination findings of [disease name] include [finding 1], [finding 2], and [finding 3].
*[[Pallor]]<ref name="MatsonYang2019">{{cite journal|last1=Matson|first1=Daniel R.|last2=Yang|first2=David T.|title=Autoimmune Lymphoproliferative Syndrome: An Overview|journal=Archives of Pathology & Laboratory Medicine|volume=144|issue=2|year=2019|pages=245–251|issn=0003-9985|doi=10.5858/arpa.2018-0190-RS}}</ref><ref name="RaoOliveira2011">{{cite journal|last1=Rao|first1=V. Koneti|last2=Oliveira|first2=João Bosco|title=How I treat autoimmune lymphoproliferative syndrome|journal=Blood|volume=118|issue=22|year=2011|pages=5741–5751|issn=0006-4971|doi=10.1182/blood-2011-07-325217}}</ref>
*[[Rash]]
*[[Petechiae]]
*[[Icterus]]
*[[Mucocutaneous]] [[bleeding]]


OR
===Head===


The presence of [finding(s)] on physical examination is diagnostic of [disease name].
*[[Lymphadenopathy]]: >90% of patients present with chronic non-malignant [[lymphadenopathy]].  It can be mild to severe, affecting multiple nodal groups.  Most commonly presents with massive non-painful hard cervical lymphadenopathy
[[File:ALPS children lymph.jpg|center|thumb|624x624px|Cervical lymphadenopathy in a child with ALPS.<ref name="SnellerWang1997">{{cite journal|last1=Sneller|first1=Michael C.|last2=Wang|first2=Jin|last3=Dale|first3=Janet K.|last4=Strober|first4=Warren|last5=Middelton|first5=Lindsay A.|last6=Choi|first6=Youngnim|last7=Fleisher|first7=Thomas A.|last8=Lim|first8=Megan S.|last9=Jaffe|first9=Elaine S.|last10=Puck|first10=Jennifer M.|last11=Lenardo|first11=Michael J.|last12=Straus|first12=Stephen E.|title=Clinical, Immunologic, and Genetic Features of an Autoimmune Lymphoproliferative Syndrome Associated With Abnormal Lymphocyte Apoptosis|journal=Blood|volume=89|issue=4|year=1997|pages=1341–1348|issn=1528-0020|doi=10.1182/blood.V89.4.1341}}</ref>]]<br />


OR
===Lungs===
*[[Pulmonary examination]] of [[Patient|patients]] with ALPS is usually normal.
===Heart===
*[[Cardiovascular]] [[examination]] of [[Patient|patients]] with ALPS is usually normal.


The presence of [finding(s)] on physical examination is highly suggestive of [disease name].
===Abdomen===
==Physical Examination==
===Skin===
* [[Pallor]]
* [[Rash]]
* [[Petechiae]]


===Head===
*[[Splenomegaly]]: >80% of patients present with clinically identifiable splenomegaly.  It can be massive.
* [[Lymphadenopathy]]: >90% of patients present with chronic non-malignant [[lymphadenopathy]].  It can be mild to severe, affecting multiple nodal groups. Most commonly presents with massive non-painful hard cervical lymphadenopathy
*[[Hepatomegaly]]: 30-40% of patients have enlarged livers.


===Abdomen===
===Back===
* [[Splenomegaly]]: >80% of patients present with clinically identifiable splenomegaly. It can be massive.
*[[Human back|Back]] [[examination]] of [[Patient|patients]] with ALPS is usually normal.
* [[Hepatomegaly]]: 30-40% of patients have enlarged livers.
===Genitourinary===
*[[Genitourinary system|Genitourinary]] [[examination]] of [[Patient|patients]] with ALPS is usually normal.
===Neuromuscular===
*[[Neuromuscular]] [[examination]] of [[Patient|patients]] with ALPS is usually normal.
===Extremities===
*[[Limb (anatomy)|Extremities]] [[examination]] of [[Patient|patients]] with ALPS is usually normal.


==References==
==References==
{{reflist|2}}
{{reflist|2}}
[[Category:Needs content]]


[[Category:Disease]]
[[Category:Disease]]
[[Category:Hematology]]
[[Category:Hematology]]
{{WH}}
{{WS}}

Latest revision as of 02:26, 11 August 2021

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Editor-In-Chief: Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] David Teachey, MD [2] Associate Editor(s)-in-Chief: Sharmi Biswas, M.B.B.S

Overview

Common physical examination findings of Autoimmune lymphoproliferative syndrome(ALPS) include lymphadenopathy, Hepatomegaly, or splenomegaly. The majority of patients(80%) have enlarged, palpable, non -tender lymph nodes for an extended period of time. Cervical, axillary, inguinal lymphadenopathy are mostly found. But preauricular, submental, epitrochlear, mediastinal, and retroperitoneal nodes are detected occasionally. Moderate to massive splenomegaly is evident in 85% of patients with ALPS. Minor hepatomegaly is also a common finding. Lymphadenopathy, splenomegaly, hepatomegaly improve with age.

Physical Examination

Skin

Head

  • Lymphadenopathy: >90% of patients present with chronic non-malignant lymphadenopathy. It can be mild to severe, affecting multiple nodal groups. Most commonly presents with massive non-painful hard cervical lymphadenopathy
Cervical lymphadenopathy in a child with ALPS.[3]


Lungs

Heart

Abdomen

  • Splenomegaly: >80% of patients present with clinically identifiable splenomegaly. It can be massive.
  • Hepatomegaly: 30-40% of patients have enlarged livers.

Back

Genitourinary

Neuromuscular

Extremities

References

  1. Matson, Daniel R.; Yang, David T. (2019). "Autoimmune Lymphoproliferative Syndrome: An Overview". Archives of Pathology & Laboratory Medicine. 144 (2): 245–251. doi:10.5858/arpa.2018-0190-RS. ISSN 0003-9985.
  2. Rao, V. Koneti; Oliveira, João Bosco (2011). "How I treat autoimmune lymphoproliferative syndrome". Blood. 118 (22): 5741–5751. doi:10.1182/blood-2011-07-325217. ISSN 0006-4971.
  3. Sneller, Michael C.; Wang, Jin; Dale, Janet K.; Strober, Warren; Middelton, Lindsay A.; Choi, Youngnim; Fleisher, Thomas A.; Lim, Megan S.; Jaffe, Elaine S.; Puck, Jennifer M.; Lenardo, Michael J.; Straus, Stephen E. (1997). "Clinical, Immunologic, and Genetic Features of an Autoimmune Lymphoproliferative Syndrome Associated With Abnormal Lymphocyte Apoptosis". Blood. 89 (4): 1341–1348. doi:10.1182/blood.V89.4.1341. ISSN 1528-0020.