Paroxysmal nocturnal hemoglobinuria laboratory findings: Difference between revisions

Jump to navigation Jump to search
VisualWikitext
(Created page with "__NOTOC__ {{ Paroxysmal nocturnal hemoglobinuria }} {{CMG}} ==Overview== ==Laboratory Findings== A sugar or sucrose lysis test, in which a patient's red blood cells are plac...")
 
No edit summary
 
(8 intermediate revisions by 5 users not shown)
Line 1: Line 1:
__NOTOC__
__NOTOC__
{{ Paroxysmal nocturnal hemoglobinuria }}
{{Paroxysmal nocturnal hemoglobinuria}}


{{CMG}}
{{CMG}}; {{AE}} {{AEL}}  


==Overview==
==Overview==
Laboratory findings consistent with the diagnosis of paroxysmal nocturnal hemoglobinuria include low hemoglobin level, low RBCs count, and negative coombs test. Flow cytometry is the diagnostic test of choice where it shows GPI deficient RBCs.
==Laboratory Findings==
==Laboratory Findings==
A sugar or sucrose lysis test, in which a patient's red blood cells are placed in low ionic strength solution and observed for hemolysis, is used for screening. A more specific test for PNH, called ''Ham's acid hemolysis'' test, is performed if the sugar test is positive for hemolysis.<ref>Ham TH. Chronic haemolytic anaemia with paroxysmal nocturnal haemoglobinuria: study of the mechanism of haemolysis in relation to acid-base equilibrium. ''[[N Engl J Med]]'' 1937;217:915-918.</ref> In a positive sucrose lysis test ionic strength facilitates the complement binding whereas in a positive Ham acid hemolysis test acidic strength facilitates the complement binding.  The differential diagnosis of a positive sugar lysis test includes some autoimmune hemolytic anemias; even leukemias can give a false positive result.  The differential diagnosis for a positive Ham test includes congenital dyserythropoietic anemia; note that a negative Ham test doesn't rule out PNH.  These assays do not reliably quantitate the percentage of PNH cells and can be falsely negative in patients who have received red blood cell transfusions. Occasionally the characteristic complement-sensitive erythrocytes cannot be demonstrated in patients with well-established PNH. This probably occurs when the production of PNH cells is relatively low and most of the PNH cells that have been made have already been destroyed either in the marrow or in the circulation.  Therefore a single normal sucrose hemolysis test cannot be considered absolute evidence that a patient does not have PNH.  
 
Laboratory findings consistent with the diagnosis of PNH include:
* Due to hemolysis:
*# Low hemoglobin
*# Low RBC count
*# Increased reticulocyte count
*# Increased LDH
*# Increased bilirubin
*# Decreased haptoglobin
*# Haemoglobinuria
*# Negative coombs test  
 
* Due to haemoglobinuria:
*# Urinalysis: positive dipstick for heme with negative microscopic analysis for RBC
*# Increased creatinine
*# Increased BUN
*# Iron deficiency: low iron, low ferritin, increased transferrin or TIBC
 
* Due to thrombosis:
*# Thrombocytopenia
*# Elevated liver function test
 
*Flow cytometry is the diagnostic study of choice to diagnose PNH. In PNH, flow cytometry shows decreased expression of GPI anchor on the RBCs surfaces.<ref name="pmid25237200">{{cite journal| author=Brodsky RA| title=Paroxysmal nocturnal hemoglobinuria. | journal=Blood | year= 2014 | volume= 124 | issue= 18 | pages= 2804-11 | pmid=25237200 | doi=10.1182/blood-2014-02-522128 | pmc=4215311 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25237200  }} </ref>
 
*Other diagnostic studies not required for diagnosis include:
** Bone marrow biopsy in cases of pancytopenia it may show hypocellular or normocellular marrow.
 
==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
[[Category:Disease]]
[[Category:Hematology]]
[[Category:Rare diseases]]
[[Category:Genetic disorders]]
[[Category:Mature chapter]]


{{WH}}
{{WH}}
{{WS}}
{{WS}}
[[Category: (name of the system)]]

Latest revision as of 20:02, 18 December 2018

Paroxysmal nocturnal hemoglobinuria Microchapters

Home

Patient Information

Overview

Historical Perspective

Classification

Pathophysiology

Causes

Differentiating Paroxysmal nocturnal hemoglobinuria from other Diseases

Epidemiology and Demographics

Risk Factors

Screening

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Electrocardiogram

X Ray

CT

MRI

Echocardiography or Ultrasound

Other Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

Cost-Effectiveness of Therapy

Future or Investigational Therapies

Case Studies

Case #1

Paroxysmal nocturnal hemoglobinuria laboratory findings On the Web

Most recent articles

Most cited articles

Review articles

CME Programs

Powerpoint slides

Images

American Roentgen Ray Society Images of Paroxysmal nocturnal hemoglobinuria laboratory findings

All Images
X-rays
Echo & Ultrasound
CT Images
MRI

Ongoing Trials at Clinical Trials.gov

US National Guidelines Clearinghouse

NICE Guidance

FDA on Paroxysmal nocturnal hemoglobinuria laboratory findings

CDC on Paroxysmal nocturnal hemoglobinuria laboratory findings

Paroxysmal nocturnal hemoglobinuria laboratory findings in the news

Blogs on Paroxysmal nocturnal hemoglobinuria laboratory findings

Directions to Hospitals Treating Paroxysmal nocturnal hemoglobinuria

Risk calculators and risk factors for Paroxysmal nocturnal hemoglobinuria laboratory findings

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmed Elsaiey, MBBCH [2]

Overview

Laboratory findings consistent with the diagnosis of paroxysmal nocturnal hemoglobinuria include low hemoglobin level, low RBCs count, and negative coombs test. Flow cytometry is the diagnostic test of choice where it shows GPI deficient RBCs.

Laboratory Findings

Laboratory findings consistent with the diagnosis of PNH include:

  • Due to hemolysis:
    1. Low hemoglobin
    2. Low RBC count
    3. Increased reticulocyte count
    4. Increased LDH
    5. Increased bilirubin
    6. Decreased haptoglobin
    7. Haemoglobinuria
    8. Negative coombs test
  • Due to haemoglobinuria:
    1. Urinalysis: positive dipstick for heme with negative microscopic analysis for RBC
    2. Increased creatinine
    3. Increased BUN
    4. Iron deficiency: low iron, low ferritin, increased transferrin or TIBC
  • Due to thrombosis:
    1. Thrombocytopenia
    2. Elevated liver function test
  • Flow cytometry is the diagnostic study of choice to diagnose PNH. In PNH, flow cytometry shows decreased expression of GPI anchor on the RBCs surfaces.[1]
  • Other diagnostic studies not required for diagnosis include:
    • Bone marrow biopsy in cases of pancytopenia it may show hypocellular or normocellular marrow.

References

  1. ↑ Brodsky RA (2014). "Paroxysmal nocturnal hemoglobinuria". Blood. 124 (18): 2804–11. doi:10.1182/blood-2014-02-522128. PMC 4215311. PMID 25237200.

Template:WH Template:WS

Retrieved from "https://www.wikidoc.org/index.php?title=Paroxysmal_nocturnal_hemoglobinuria_laboratory_findings&oldid=1510497"