Mr. Hematologist we want a differential diagnosis.

PNH
diagnosis suggested by characteristic intermittent haemoglobinuria and demonstrastion of haemosiderin in urine,and confirmed by positive serological test.
1/hams acid serum test
2/sucrose haemolysis test

مساء الخير للجميع وللمشرف العظيم وقدوة المعيدين أ.اشرف شقليه
الحالة تمثلPNH
FOR DIAGNOSIS:
}CBC SHOW LOW HB{
high LDH, high RETIC count, high bilirubin,
DAT test is NEGATIVE
FLOW CYTMETRY FOR CD55&CD59
HAMS TEST

hellow
Dr.Ashraf i've Questions :
*does he have hepatosplenomegaly?
*does he take any medications?
*are there an abnormal cells in the periphral blood?
*does the marrow show hypoplasia?
*Ham test result?
:sm200::sm202:

Thank you professor Ashraf for refreshing our memories, i realy admire your efforts and knowledge,you sure earned all the love and respect in this site...
opening and twisting my mind i couldn't think in many conditions...PNH is a perfect diagnosis that can be confirmed by flowcytometry for CD55 and CD59 usually markedly diminished or absent together with other tests like the Ham's test , i would like also to do a hemoglobin electrophoresis for this case or at least sickling test to exclude sickle cell anemia and that is my second differential diagnosis...
i could not recall any other situation that with a combined panacytopenia,intravascular hemolysis and thrombosis except for malignancy and that is my third differential diagnosis.
thank you again and waiting for your remarks...

I am very glad to hear such nice, cute and sympathatic words from

Case solution

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Hemoglobin electrophoresis shows normal migration pattern, sickling test is negative
No signs or sysmptoms of a malignancy and cancer markers are all negative
The case really represents PNH
What is characteristic in PNH is the thrombosis in unusual sites
Laboratory diagnosis of PNH
Screening laboratory tests relevant to PNH include a complete blood count (CBC), stained blood smear, and bone marrow examination. Leukocyte alkaline phosphatase (LAP) is usually decreased in PNH.
The classic and highly specific way to identify the RBC PNH clone is Ham's test. Only one other disease is associated with a positive Ham's test: hereditary erythroid multinuclearity with positive acidified serum (HEMPAS), distinguished from PNH by a negative sucrose hemolysis test. Hence, Ham's test is highly specific for PNH. The Ham's test is based on the precept that complement will attach to RBCs at low pH and that PNH RBCs are more sensitive to complement. Whole defibrinated blood collected in heparin is utilized. Cells from the patient and controls are tested for hemolysis with (1) unmodified serum, (2) serum, pH 6.8, (3) heat-inactivated serum (55°C for 3 min.), and (4) heated serum with guinea pig complement. Positivity should be encountered with: (1) PNH cells only and (2) acidified serum; (3) destroying complement (heating) prevents the hemolysis, and (4) not with guinea pig complement
The sucrose lysis test is another standard screening test for PNH. Low ionic strength isotonic sucrose induces serum globulin to bind complement on the RBC membrane.
Flow cytometry (FCM) became the definitive test for PNH. Usually, CD55 and CD59 are both measured by FCM. Depressed levels of both of these glycoproteins are consistent with PNH [/LEFT]

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