Pancytopenia

Pancytopenia is a medical condition in which there is a reduction in the number of red and white blood cells, as well as platelets.

If only two parameters from the full blood count are low, the term bicytopenia can be used. The diagnostic approach is the same as for pancytopenia.

Definition

• anemia: hemoglobin < 13.5 g/dL (male) or 12 g/dL (female).
• leukopenia: total white cell count < 4.0 x 10⁹/L. Decrease in all types of white blood cells (revealed by doing a differential count).
• thrombocytopenia: platelet count < 150×10⁹/L.

The disease is marked by an inappropriate and ineffective T cell activation that leads to an increased hemophagocytic activity. The T cell activated macrophages engulf erythrocytes, leukocytes, platelets, as well as their progenitor cells. Such finding is common in the syndrome, which is also referred to as hemophagocytic lymphohistiocytosis (HLH). Along with pancytopenia, HLH is characterized by fever, splenomegaly, and hemophagocytosis in bone marrow, liver, or lymph nodes.

Iatrogenic causes of pancytopenia include chemotherapy for malignancies if the drug or drugs used cause bone marrow suppression. Rarely, drugs (antibiotics, blood pressure medication, heart medication) can cause pancytopenia. The antibiotics Linezolid and Chloramphenicol can cause pancytopenia in some individuals.

Rarely, pancytopenia may have other causes, such as mononucleosis, or other viral diseases. Increasingly, HIV is itself a cause for pancytopenia.

Causes

• Familial hemophagocytic syndrome
• Aplastic anemia
• Gaucher's disease
• metastatic carcinoma of bone
• multiple myeloma
• overwhelming infections
• lymphoma
• myelofibrosis
• Dyskeratosis congenita
• Myelodysplastic syndrome
• Leukemia
• Leishmaniasis
• Severe Folate or vitamin B12 deficiency
• Systemic lupus erythematosus
• Paroxysmal nocturnal hemoglobinuria (blood test)
• viral infections (such as HIV, EBV--undetermined virus is most common).
• Alimentary toxic aleukia
• Copper deficiency
• Pernicious anemia
• Medication
• Hypersplenism
• Osteopetrosis
• Organic acidurias (Propionic Acidemia, Methylmalonic Aciduria, Isovaleric Aciduria)
• Low dose arsenic poisoning
• Sako disease (Myelodysplastic-cytosis)
• Chronic radiation sickness^[1]
• LIG4 syndrome

Diagnosis

Pancytopenia usually requires a bone marrow biopsy in order to distinguish among different causes.

References

External links

Wikimedia Commons has media related to Hemophagocytic Infections and Syndromes.

• EID Journal (Volume 6, Number 6), CDC, December 2000.
• Aetiopancyto – Université Angers