Pancytopenia
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| Pancytopenia | |
|---|---|
| Specialty | Hematology |
Pancytopenia is a
If only two parameters from the complete blood count are low, the term bicytopenia can be used. The diagnostic approach is the same as for pancytopenia.
Causes
Iatrogenic causes of pancytopenia include chemotherapy for malignancies if the drug or drugs used cause
Rarely, pancytopenia may have other causes, such as mononucleosis or other viral diseases. Increasingly, HIV is itself a cause of pancytopenia.[2]
- 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; an 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[3]
- LIG4 syndrome
Mechanism
The mechanism of pancytopenia involves either haemopoiesis itself, decreasing blood cell productions in number (aplastic anemia), haemopoietic stem cells are displaced by malignant cells (Leukemia, lymphoma, MDS) or they are being pooled (sequestrated) (spleen)/destroyed (immune) outside bone marrow. The mechanisms for pancytopenia differ according to the etiology. For example, in hemophagocytic lymphohistiocytosis (HLH) there is marked 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. Along with pancytopenia, HLH is characterized by fever, splenomegaly, and hemophagocytosis in bone marrow, liver, or lymph nodes.[4]
Diagnosis
Pancytopenia usually requires a
- 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.
Treatment
Treatment is done to address the underlying cause. To tide over immediate crisis Blood transfusion with packed red blood cells (PRBC) or platelet transfusion may be done. Sometimes there are obvious clinical clues to suggest underlying B12 deficiency for a cause of pancytopenia.[6] In this selected cases even with severe anemia blood product transfusions can be avoided and vitamin B12 treatment itself suffice.[7] In other situations like acute leukemia, Myelodysplastic syndrome, aplastic anemia etc. disease specific therapy is needed.[8]
References
- ISBN 978-0-12-386455-0, archivedfrom the original on 2022-02-14, retrieved 2020-12-11
- PMID 24238033.
- ^ Kossenko MM, Akleyev AA, Degteva MO, Kozheurov VP, Degtyaryova RC (August 1994). "Analysis of Chronic Radiation Sickness Cases in the Population of the Southern Urals (AD-A286 238)" (PDF). DTIC. p. 5. Archived from the original on 8 October 2012. Retrieved 1 August 2013.
Complete blood counts, when taken, revealed pancytopenia.
- PMID 21900192.
- ^ "Rudhiram Hematology Clinic". www.rudhiramhematology.com. Archived from the original on 15 March 2022. Retrieved 2 February 2022.
- S2CID 5772424.
- S2CID 5772424.
- from the original on 2022-02-03. Retrieved 2022-02-02.
External links
- EID Journal (Volume 6, Number 6), CDC, December 2000.
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