1. A 24- year- old woman presented to her primary-care physician for evaluation of new tea –colored urine noticed intermittently over the past five days . Her last menstrual cycle was two weeks ago ,and they have been occurring regularly

2. There is no history of renal stones , NSAID abuse, weight loss, night sweats ,fever , melena , or hemoptysis .her vital signs were unremarkable . on initial evaluation , CBC,urinalysis,and renal ultrasound were normal and urine pregnancy test was negative.

3. A few days later , she developed jaundice with abdominal pain
A few days later , she developed jaundice with abdominal pain . Repeat testing at that time showed the following ; WBC count 3600/mm3 ,hemoglobin 4 g/dl, platelet count 189,000/mm3 , MCV 75 fl , RDW 28 ,

4. Reticulocyte count 10. 9% (N. V. =0. 5- 2
Reticulocyte count 10.9% (N.V.= % ) ,Total serum bilirubin 7.5mg/dl (N.V.= mg/dl), indirect bilirubin 5.5 mg/dl (N.V.= mg/dl) , AST 213 U/L serum LDH 1500 U/L (N.V.= ), serum haptoglobin 10 mg/dl .

5. The urinalysis showed hemoglobinuria. Direct coomb test is negative
The urinalysis showed hemoglobinuria . Direct coomb test is negative. Liver ultrasound shows mild hepatomegaly and no signs of stones , biliary ductal dilatation , or hepatic mass . Flow cytometry of peripheral blood showed absent expression of CD55 and CD59 on 78% of red blood cells .

6. What do you think is the most appropriate drug to use in this condition ?

7. Eculizumab. Because it is a humanized monoclonal antibody that binds to the C5 component of complement and inhibits terminal complement activation on the red cell surface .

8. What does occur in PNH?

9. is an acquired clonal disorder, which arises following a somatic mutation in a multipotential stem cell

10. What is characteristics of PNH ?

11. RBC’s are susceptible to the lytic action of complement.
Chronic but episodic intravascular haemolysis.
Occurs mainly during sleep.
Moderately to severe panhypoplasia.
Neutrophil dysfunction.
Renal insufficiency.
Dysphagia.
Tendency to venous thrombosis.

12. Classification of PNH ?

13. 1- PNH I red cells are normal with regard to their sensitivity to complement-mediated lysis.
2- PNH II red cells show a moderately increased sensitivity.
3- PNH III red cells show a markedly increased sensitivity.

14. What is the clinical picture?

15. Episodes of intravascular haemolysis.
Haemoglobinuria.
Haemorrhage.
Infection.
Thrombotic complications.

16. What is Triggering factors for haemolysis ?

17. Mostly is unknown.
Infection.
Vaccination.
Blood transfusion.
Menstrual cycle.