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Paroxysmal Nocturnal Hemoglobinuria (PNH), High Sensitivity, RBC and WBC (8000101388)
Test Mnemonic:

PNH
Specimen Requirements:
Collection:

Routine venipuncture; Collect in EDTA (lavender tube).   The minimum volume required is 90% of the stated volume
Container:

4.0 ml EDTA (lavender top)
Minimum Volume:

1.0 ml
Storage/Transport:

Sample delivered to Hematopathology lab and are stored at 2-8C up to 72 hours
Stability:

Sample stable for 72 hours at 2-8C
Causes for Rejection:
  1. Clotted sample
  2. Hemolyzed sample
  3. Old sample >72 hours old
  4. Improperly store sample
Reference Range:

RBC: 0.00-0.002%

WBC-Granulocytes: 0.00-0.002%

WBC-Monocytes: 0.00-0.005%
Turnaround Time:

72 hours
Methodology:

Paroxysmal nocturnal hemoglobinuria (PNH) is a condition that causes hemolytic anemia, bone marrow failure, and a proclivity to develop thrombosis. PNH occurs when the PIG-A gene of bone marrow stem cells undergoes mutation. Stem cells develop into RBCs, WBCs, platelets, and other mature cells that contain the mutated PIG-A gene, and as a result, lack sufficient number of glycosylphosphatidylinositol-anchored protein (GPI-AP). The GPI-APs have various roles, some of which to help to prevent RBC destruction, fight infection, and promote thrombosis CD markers (CD14, CD16, CD24, and CD59) are GPI-linked proteins that are used as markers for the presence of GPI, as is fluorescent aerolysin (FLAER), which binds to GPI-APs. Patients with PNH lack sufficient GPIs and therefore have reduced expression of these cell markers and FLAER compared to patients without PNH. Patients with complete loss of CD59 are more likely to have hemolysis compared to patients with partial loss of CD59.
Performed:
Lab:

Hematopathology Division-Flow Cytometry Section.
Synonyms:

PNH, CD59, FLAER, CD24, CD15, CD14, CD64, PI-Linked Antigens.
Clinical Indication:

Diagnose PNH in patients with the following clinical indications:

  • Hemoglobinuria
  • Unexplained hemolysis with iron deficiency, abdominal pain, thrombosis, dysphagia, or granulocytopenia/thrombocytopenia
  • Coombs-negative hemolytic anemia that cannot be otherwise explained
  • Thrombosis at unusual sites or with unexplained hemolytic anemia or cytopenia
  • Evidence of bone marrow failure (aplastic anemia, hypoplastic anemia, MDS)

Monitor disease progression and response to treatment in patients with confirmed PNH
CPT 4 Code:

88187,88184,88185 x 6.
When ordering tests for which Medicare or Medicaid reimbursement will be sought, physicians should only order tests that are medically necessary for the diagnosis or treatment of the patient. Components of the organ or disease panels may be ordered individually. The diagnostic information must substantiate all tests ordered and must be in the form of an ICD-10 code or its verbal equivalent.
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