Autoimmune lymphoproliferative syndrome physical examination: Difference between revisions
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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}} | ||
==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=== | |||
*[[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]] | |||
===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 | |||
[[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 /> | |||
===Lungs=== | |||
*[[Pulmonary examination]] of [[Patient|patients]] with ALPS is usually normal. | |||
===Heart=== | |||
*[[Cardiovascular]] [[examination]] of [[Patient|patients]] with ALPS is usually normal. | |||
===Abdomen=== | |||
== | |||
== | |||
*[[Splenomegaly]]: >80% of patients present with clinically identifiable splenomegaly. It can be massive. | |||
* [[ | *[[Hepatomegaly]]: 30-40% of patients have enlarged livers. | ||
=== | ===Back=== | ||
* [[ | *[[Human back|Back]] [[examination]] of [[Patient|patients]] with ALPS is usually normal. | ||
* [[ | ===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:Disease]] | [[Category:Disease]] | ||
[[Category:Hematology]] | [[Category:Hematology]] | ||
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
Lungs
- Pulmonary examination of patients with ALPS is usually normal.
Heart
- Cardiovascular examination of patients with ALPS is usually normal.
Abdomen
- Splenomegaly: >80% of patients present with clinically identifiable splenomegaly. It can be massive.
- Hepatomegaly: 30-40% of patients have enlarged livers.
Back
- Back examination of patients with ALPS is usually normal.
Genitourinary
- Genitourinary examination of patients with ALPS is usually normal.
Neuromuscular
- Neuromuscular examination of patients with ALPS is usually normal.
Extremities
- Extremities examination of patients with ALPS is usually normal.
References
- ↑ 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.
- ↑ 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.
- ↑ 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.