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
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Hereditary spherocytosis Microchapters |
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Differentiating Hereditary spherocytosis from other Diseases |
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Diagnosis |
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Treatment |
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Case Studies |
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Hereditary spherocytosis classification On the Web |
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American Roentgen Ray Society Images of Hereditary spherocytosis classification |
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Risk calculators and risk factors for Hereditary spherocytosis classification |
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 |