Tuesday, August 11, 2015

Pancytopenia

Today we discussed a very interesting case of pancytopenia. This is a great internal medicine topic that is definitely worth reviewing. The causes of pancytopenia are extensive so it can be overwhelming to come up with a comprehensive differential diagnosis. That's why it's great to have an organized approach and stick to it every time!

One approach that we discussed is to divide the causes of pancytopenia into those associated with bone marrow which is either hypocellular or hypercellular:

Hypocellular Marrow:
  • Drugs/Toxins: Methotrexate, chemotherapeutics, various antibiotics, allopurinol...
  • Infection: Parvovirus B19, EBV, HIV, TB, Hepatitis, Sepsis 
  • Autoimmune: Lupus, HLH
  • Paroxysmal Nocturnal Hemoglobinuria (PNH)
Hypercellular Marrow
  • Malignancy: Leukemia, Lymphoma, MDS
  • Nutritional: Deficiencies in B12, folate, copper
  • Marrow Replacement: Myelofibrosis, Granulomatous disease (TB, sarcoid, fungal), solid tumor metastatic disease
A few words about paroxysmal nocturnal hemoglobinuria (PNH):
This is a rare acquired stem cell disorder which can present as unexplained hemolytic anemia, thrombosis, hemoglobinuria and non-specific symptoms. It can certainly present as pancytopenia along with the associated complications. PNH should be considered in Coombs negative hemolytic anemia or other unexplained cases of anemia. The diagnosis is made with flow cytometry using a technique that determines whether certain cell wall proteins (GPI) are present or not - they are absent in PNH. Definitive treatment of PNH is a stem cell transplant.

Another few words about Myelophthisic Anemia:
What a great word! Whoever decided to put a `ph` next to a `th` must have enjoyed watching people struggle with pronouncing things... This entity was brought  up today in morning report. Myelophthisis refers to bone marrow being displaced by some other process. This could include tumor, infection (TB), granulomatous conditions (sarcoid) or fibrosis. There are often clues on the peripheral blood film that may suggest a myelophthisic process. These include teardrop cells and nucleated red blood cells.

Check out this article for a general overview of the causes of pancytopenia: http://www.ncbi.nlm.nih.gov/pubmed/23270895 

12 comments :

Cookie Walker said...


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willy same said...

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JOY BEN said...

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Timothy Eric said...

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Varun said...

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