Hereditary spherocytosis classification: Difference between revisions

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| colspan="1" rowspan="1" |Mild -moderate 3
| colspan="1" rowspan="1" |Mild -moderate 3
| colspan="1" rowspan="1" |1 moderately severe case described
| colspan="1" rowspan="1" |1 moderately severe case described
|}
* HS also classified on basis of severity as mentioned on the below table.
{| class="wikitable"
|-
! colspan="1" rowspan="1" |Severity
! colspan="1" rowspan="1" |Hgb (g/dL)
! colspan="1" rowspan="1" |Reticulocytes (%)
! colspan="1" rowspan="1" |Splenectomy
|-
| colspan="1" rowspan="1" |'''Mild'''
| colspan="1" rowspan="1" |11-15
| colspan="1" rowspan="1" |3-8
| colspan="1" rowspan="1" |Not necessary
|-
| colspan="1" rowspan="1" |'''Moderate'''
| colspan="1" rowspan="1" |8-11.5
| colspan="1" rowspan="1" |>8
| colspan="1" rowspan="1" |Consider if activity level & quality of life are decreased
|-
| colspan="1" rowspan="1" |'''Moderately severe'''
| colspan="1" rowspan="1" |6-8
| colspan="1" rowspan="1" |≥10
| colspan="1" rowspan="1" |Indicated at age >5 yrs
|-
| colspan="1" rowspan="1" |'''Severe'''
| colspan="1" rowspan="1" |<6
| colspan="1" rowspan="1" |≥10
| colspan="1" rowspan="1" |Indicated at age >3 yrs
|-
| colspan="1" rowspan="1" |'''Normal 1'''
| colspan="1" rowspan="1" |11.7-15.7 (adult females) 13.3-17.7 (adult males)
| colspan="1" rowspan="1" |0.5-1.5 2
|}
|}



Revision as of 19:31, 29 July 2018

Hereditary spherocytosis Microchapters

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief:

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Overview

Classification

SPH1 ANK1 Ankyrin-1 AD Mild -moderate
AR Moderately severe -severe Often transfusion dependent
SPH2 SPTB Spectrin beta chain, erythrocytic AD Mild -moderate
AR Severe 1 fatal infantile case described
SPH3 SPTA1 Spectrin alpha chain, erythrocytic 1 AR Severe Transfusion dependent
SPH4 SLC4A1 Band 3 (anion transport protein) AD Mild -moderate Certain SLC4A1 pathogenic variants cause disease only when biallelic.
SPH5 EPB42 Protein 4.2 2 AR Mild -moderate 3 1 moderately severe case described
  • HS also classified on basis of severity as mentioned on the below table.
Severity Hgb (g/dL) Reticulocytes (%) Splenectomy
Mild 11-15 3-8 Not necessary
Moderate 8-11.5 >8 Consider if activity level & quality of life are decreased
Moderately severe 6-8 ≥10 Indicated at age >5 yrs
Severe <6 ≥10 Indicated at age >3 yrs
Normal 1 11.7-15.7 (adult females) 13.3-17.7 (adult males) 0.5-1.5 2

References

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