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==References==
==References==
{{Reflist|2}}
== Differentiating between Hemoglobinopathies ==
 
{{WH}}
{{WS}}
[[Category: (name of the system)]]


{| class="wikitable"
|+
! colspan="4" rowspan="2" |
! rowspan="2" |'''Gene type'''
! colspan="4" |'''Red blood cell (RBC) count g/dl'''
! rowspan="2" |'''Hemoglobin pattern'''
! rowspan="2" |Differentiating Symptoms
|-
!Hemoglobin g/dl
!MCH /pg
! colspan="2" |Hemoglobinpattern
|-
| colspan="4" rowspan="4" |Alpha Thalassemia
|<nowiki>-+/++</nowiki>
|Normal
|Normal
| colspan="2" |Normal
|Normal
|None
|-
| -+/-+
--/++
|Normal or low
|<26
| colspan="2" |Normal
|Normal
|Mild anemia
|-
| --/-+
|8 to 10
|<22
| colspan="2" |HbH &asymp
10 to 20%         
|HbH 10 to 20%
|Chronic hemolytic anemia
|-
|Hb Bart’s hydrops fetalis
--/--
|<6
|<20
| colspan="2" |
* Hb Bart’s 80 to 90%,
* Hb Portland &amp;asymp; 10 to 20%,
* HbH <1%
|Hb Bart’s 80 to 90%,
Hb Portland 10 to 20%,


HbH  <1%
|Life-threatening fetal anemia
|-
| rowspan="7" |β-thalassemia
| colspan="3" rowspan="3" |Heterozygous
|&β'''/'''++
|9 to 15
|
| colspan="2" |HbA2 >3.2%
|
|
|-
|&β'''/'''+-
|
|
| colspan="2" |HbF 0.5 to 6%
|
|
|-
|&β/--
|
|19 to 25
| colspan="2" |
|
|
|-
| colspan="3" |Compound heterozygous
|'''&amp;beta;''' + /'''&amp;beta;''' 0
|
|
|
|
|
|
|-
| colspan="3" rowspan="3" |Homozygous
|'''&amp;beta;'''+/'''&amp;beta;'''+
|<7
|
|
|
|
|
|-
|'''&amp;beta;''' 0 /'''&amp;beta;''' 0
|
|
|
|
|
|
|-
|
|
|
|
|
|
|
|-
|Sickle cell Disease
|
|
|
|
|6 to 9
|
|
|
|
|
|-
|HBC
|
|
|
|
|
|
|
|
|
|
|}


==Pathophysiology==
==Pathophysiology==

Revision as of 18:07, 7 September 2018


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

Overview

Differentiating [disease name] from other diseases on the basis of [symptom 1], [symptom 2], and [symptom 3]

On the basis [symptom 1], [symptom 2], and [symptom 3], [disease name] must be differentiated from disseminated intravascular coagulation (DIC) , thrombotic thrombocytopenic purpura (TTP),systemic vasculitis , [disease 4], [disease 5], and [disease 6].

Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
Symptoms Physical examination
Lab Findings Occur Histopathology
Diarrhea Abdominal pain decrease urin Physical exam 1 Physical exam 2 fd CBC PC PT PTT FDP D-dimer LDH haptoglobin Coombs test PBS BUN Cr S/C Pediatric Adult Imaging 3
Disseminated intravascular coagulation (DIC) NL/_
Hemolytic uremic syndrome Hemolitic anemia NL NL NL +++ +
Thrombotic thrombocytopenic purpura (TTP) + +++
Systemic vasculitis
Diseases Symptom 1 Symptom 2 Symptom 3 Physical exam 1 D Physical exam 3 Lab 1 Lab 2 Lab 3 Imaging 1 Imaging 2 Imaging 3 Histopathology Gold standard Additional findings
Differential Diagnosis 4
Differential Diagnosis 5
Differential Diagnosis 6

References

Differentiating between Hemoglobinopathies

Gene type Red blood cell (RBC) count g/dl Hemoglobin pattern Differentiating Symptoms
Hemoglobin g/dl MCH /pg Hemoglobinpattern
Alpha Thalassemia -+/++ Normal Normal Normal Normal None
-+/-+

--/++

Normal or low <26 Normal Normal Mild anemia
--/-+ 8 to 10 <22 HbH &asymp

10 to 20%

HbH 10 to 20% Chronic hemolytic anemia
Hb Bart’s hydrops fetalis

--/--

<6 <20
  • Hb Bart’s 80 to 90%,
  • Hb Portland &asymp; 10 to 20%,
  • HbH <1%
Hb Bart’s 80 to 90%,

Hb Portland 10 to 20%,

HbH <1%

Life-threatening fetal anemia
β-thalassemia Heterozygous /++ 9 to 15 HbA2 >3.2%
/+- HbF 0.5 to 6%
&β/-- 19 to 25
Compound heterozygous &beta; + /&beta; 0
Homozygous &beta;+/&beta;+ <7
&beta; 0 /&beta; 0
Sickle cell Disease 6 to 9
HBC

Pathophysiology

  1. The pathophysiolgy of xxx disease in unknown.
  2. But it may follow xxx pathway
    • It is believed that xxx might work on yyy to produce zz.

Pathology

  • Idiopathic
  • Itarogenic
  • cardiac

References

 
 
 
 
 
 
 
 
 
 
 
 
 
 
History & clinical symptoms
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Blood test
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
hemolysate
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Diagnosis of abnormal hemoglobins
 
 
 
 
 
 
 
 
 
 
 
Diagnisis of β-thalassemia
 
 
 
 
 
 
 
Dignisis of α-thalassemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Alkaline electrophoresis
• acid electrophoresis
•HPLC
 
 
 
 
 
 
 
 
 
 
 
Electrophoresis HPLC
• HbA2,Hbf
 
 
 
 
 
 
 
Electrophoresis HPLC
• DNA testing
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Evidence of abnormality,comparison of mobility with that of known abnormalities, if HBS suspected: solubility test
 
 
Separation/quantification
 
 
 
 
 
 
 
 
 
Evalution of all data and findings
 
 
 
 
 
 
 
Evalution of all data and findings
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Diagnisis of β-thalassemia
 
 
 
 
 
 
 
Dignisis of α-thalassemia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
DNA sequencing if needed
 
 
 
 
 
 
 
 
 
 
 
 
DNA sequencing if needed(thalassemia major, thalassemia intermedia
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Evaluation of all data and findings(including blood count ethnic and origin
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
 
Diagnosis of hemoglobinopathy