Splenic sequestration: Difference between revisions
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==Background== | ==Background== | ||
*Leading cause of pediatric mortality related to [[sickle cell disease]] | *Leading cause of pediatric mortality related to [[sickle cell disease]]<ref>Brousse V et al. Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 pediatric patients. British Journal of Haematology. 2012;156:643-648.</ref> | ||
*More common in pediatric patients as many adults with SCD have autoinfarcted their spleen | *More common in pediatric patients as many adults with SCD have autoinfarcted their spleen | ||
*Earliest life threatening complication of SCD with median age 1-4<sup>3</sup> | *Earliest life threatening complication of SCD with median age 1-4<sup>3</sup> | ||
*Red blood cells sickle, aggregate, and occlude splenic vasculature | *Red blood cells sickle, aggregate, and occlude splenic vasculature<ref>Brousse V et al. The spleen and sickle cell disease: the sick(led) spleen. British Journal of Haematology. 2014;166:165-176.</ref><ref>Lovett PB et al. Sickle Cell Disease in the Emergency Department. Emerg Med Clin N Am. 2014;32(3):629-647.</ref> | ||
*Sequestering leads to splenomegaly | *Sequestering leads to splenomegaly | ||
**Defined as splenomegaly and a 2g/dL drop in hemoglobin | **Defined as splenomegaly and a 2g/dL drop in hemoglobin | ||
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*Circulatory collapse | *Circulatory collapse | ||
*Splenomegaly | *Splenomegaly | ||
==Differential Diagnosis== | ==Differential Diagnosis== | ||
{{Sickle cell DDX}} | {{Sickle cell DDX}} | ||
==Evaluation== | ==Evaluation== | ||
[[File:PMC4800468 10.1177 2324709616638363-fig1.png|thumb|CT abdomen demonstrating enlarged spleen consistent with acute splenic sequestration crisis.]] | |||
===Workup=== | |||
*Labs | *Labs | ||
**CBC | **CBC | ||
| Line 38: | Line 32: | ||
**Reticulocyte count | **Reticulocyte count | ||
**LFTs | **LFTs | ||
**Blood cultures | **[[Blood cultures]] | ||
===Diagnosis=== | |||
*Imaging not necessary to make diagnosis | *Imaging not necessary to make diagnosis | ||
==Management== | ==Management== | ||
*Pain control | *Pain control | ||
*IV fluid resuscitation and blood transfusion | *IV fluid resuscitation and blood transfusion<ref>Abboud MR. Standard management of sickle cell disease complications. Hematology/Oncology and Stem Cell Therapy. 2020;3(1):85-90. | ||
</ref> | |||
**Caution with IVF if anemia is severe | **Caution with IVF if anemia is severe | ||
**Goal Hct 35% to avoid [[hyperviscosity syndrome]]<sup>3</sup> | **Goal Hct 35% to avoid [[hyperviscosity syndrome]]<sup>3</sup> | ||
*Treat underlying cause if found - infection common | *Treat underlying cause if found - infection common | ||
*Possible splenectomy | *Possible splenectomy | ||
**Recurrence rate is 50-75%<sup>1,2,3</sup> | **Recurrence rate is 50-75%<sup>1,2,3</sup><ref>Kane I, Nagalli S. Splenic Sequestration Crisis. [Updated 2020 Jul 6]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK553164/</ref> | ||
*Hematology consultation | *Hematology consultation | ||
==Disposition== | ==Disposition== | ||
*Admission | *Admission | ||
==Complications== | |||
*[[Shock]] | |||
*[[Splenic infarct]] | |||
*[[Splenic rupture]] | |||
*[[Sepsis]] | |||
==See Also== | ==See Also== | ||
[[Sickle cell crisis]] | *[[Sickle cell crisis]] | ||
==External Links== | ==External Links== | ||
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==References== | ==References== | ||
<references/> | <references/> | ||
[[Category:GI]] | |||
[[Category:Heme/Onc]] | |||
Latest revision as of 19:59, 12 August 2020
Background
- Leading cause of pediatric mortality related to sickle cell disease[1]
- More common in pediatric patients as many adults with SCD have autoinfarcted their spleen
- Earliest life threatening complication of SCD with median age 1-43
- Red blood cells sickle, aggregate, and occlude splenic vasculature[2][3]
- Sequestering leads to splenomegaly
- Defined as splenomegaly and a 2g/dL drop in hemoglobin
- Some resources differentiate between major and minor episodes depending on the hgb drop
- Defined as splenomegaly and a 2g/dL drop in hemoglobin
- Lifelong prevalence 7-30%3
- Often precipitated by underlying infection (pneumonia, gastroenteritis)
Clinical Features
- Abdominal pain
- Fatigue
- Pallor
- Tachycardia
- Anemia
- Hypotension
- Circulatory collapse
- Splenomegaly
Differential Diagnosis
Sickle cell crisis
- Vaso-occlusive pain crisis
- Bony infarction
- Dactylitis
- Avascular necrosis of femoral head
- Acute chest syndrome
- Asthma
- Pulmonary hypertension
- Gallbladder disease
- Acute hepatic sequestration
- Infection
- Parvovirus B19
- Splenic sequestration
- CVA
- Cerebral aneurysm and ICH
- Priapism
- Papillary necrosis
Evaluation
Workup
- Labs
- CBC
- BMP
- LDH
- Reticulocyte count
- LFTs
- Blood cultures
Diagnosis
- Imaging not necessary to make diagnosis
Management
- Pain control
- IV fluid resuscitation and blood transfusion[4]
- Caution with IVF if anemia is severe
- Goal Hct 35% to avoid hyperviscosity syndrome3
- Treat underlying cause if found - infection common
- Possible splenectomy
- Recurrence rate is 50-75%1,2,3[5]
- Hematology consultation
Disposition
- Admission
Complications
See Also
External Links
References
- ↑ Brousse V et al. Acute splenic sequestration crisis in sickle cell disease: cohort study of 190 pediatric patients. British Journal of Haematology. 2012;156:643-648.
- ↑ Brousse V et al. The spleen and sickle cell disease: the sick(led) spleen. British Journal of Haematology. 2014;166:165-176.
- ↑ Lovett PB et al. Sickle Cell Disease in the Emergency Department. Emerg Med Clin N Am. 2014;32(3):629-647.
- ↑ Abboud MR. Standard management of sickle cell disease complications. Hematology/Oncology and Stem Cell Therapy. 2020;3(1):85-90.
- ↑ Kane I, Nagalli S. Splenic Sequestration Crisis. [Updated 2020 Jul 6]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2020 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK553164/