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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 complete blood count are low, the term bicytopenia can be used. 

Systemic diseases that lead to increase circulating levels in inflammatory cytokines and hormones (interleukin+6, tumor necrosis factor alpha, hepcidin) can cause bone marrow suppression.

Exposure to a medication or toxin with  myelosuppressive properties can lead to pancytopenia.

Nutritional deficiencies can result in impaired production of blood cells: vitamin B 12, folate, copper.

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. 

Rarely, pancytopenia can be caused by mononucleosis or other viral diseases. 

Increasingly, HIV is itself a cause of pancytopenia.

Differential diagnosis:

Familial hemophagocytic syndrome

Aplastic anemia

Gaucher’s disease

Metastatic carcinoma to 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 

Viral infections (such as HIV, EBV; an undetermined virus is most common)

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

LIG4 syndrome

Pancytopenia can occur either due to mother cells that are decreased themselves in number such as aplastic anemia, mother cells are overwhelmed by malignanrt cells as with leukemia, lymphoma, MDS, or they are being sequestrated in the spleen or destroyed inside  the bone marrow. 

In the 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. 

Myelophthisis is the infiltration of bone marrow by non-hematopoietic cells leading to disruption of normal hematopoiesis or the development of bone marrow fibrosis.

Causes of myelophthisis includes solid tumors with metastasis to the bone marrow, granulomatous and lipid storage disorders and primary myelofibrosis.

Aplastic anemia can be caused by auto immune diseases, toxins, infections, or inherited bone marrow failure syndromes.

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

Treatment depends on the underlying cause.

To tide over immediate crisis transfusion with packed red blood cells (PRBC) or platelet transfusion may be done.

With B12, folate deficiency acute leukemia, Myelodysplastic syndrome, aplastic anemia, disease specific therapy is needed.

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