(All the articles published in past are available at www.shyamhemoncclinic.com)
Question: Dr. Chiragbhai, thank you for explaining in last part about treatment of aplastic anemia, including role of ATG, and role of Stem Cell Transplant. Also, for the information that Steroids have no role in treatment of aplastic anemia. After aplastic anemia articles, some readers have asked questions about how to approach a case of Pancytopenia. Can you guide us?
Ans: Sure. That is an important clinical issue. Pancytopenia (red cell, white cell, platelets all low) or Bicytopenia (any of the two cell lines are low) have essentially same differential diagnosis. Both should be approached in same manner. This used to be a difficult problem for me also before I joined Hematology. But learning systematic approach and some experience made it much easier to diagnose.
Que: So, how do you approach such a patient?
Ans: The differential diagnosis is very wide. Many conditions can lead to pancytopenia, including both acute and chronic, infectious and non infectious. So, one can approach this in many ways. One such approach is to first clinically and laboratory wise rule out common causes like B12 or folic acid deficiency OR obvious acute viral infection OR hypersplenism OR other clinically apparent or known disease from patient’s history.
Once this step is taken care of, next step is Bone Marrow Aspiration and Biopsy, with special tests as needed. Bone marrow examination is the cornerstone of diagnosis in most cases. This test divides all diseases under two major categories:
Diseases with Hypocellular marrow AND Diseases with Hypercellular marrow.
Remember, bone marrow aspiration is not sufficient, and a biopsy (so called, trephine biopsy) should also be obtained, as most cases will need that as well. Biopsy is considered gold standard for defining marrow cellularity. Aspirate can sometimes be misleading in defining cellularity. For this reason, aplastic anemia diagnosis is rarely made based on only aspirate.
If diagnosis is not clinically apparent, or after basic laboratory tests, bone marrow examination must be obtained sooner rather than later. We frequently come across patients who are undergoing several tests and imaging studies etc, but not bone marrow examination. Delaying bone marrow examination, puts patients at risk of missing serious diagnoses, and expenses of other indirect unnecessary tests. Bone marrow biopsy is an office procedure, requires only local anesthesia and minor discomfort for patient similar to a dental procedure. Patients need to be counseled, as many patients fear the word “biopsy”, and also feel that when we order biopsy, we are going to diagnose cancer. Instead, using word “bone marrow examination or test” may reduce anxiety significantly and allow patients to undergo this simple yet extremely important diagnostic test in time.
Also, many doctors delay this test when patient has pancytopenia, for fear of bleeding. Bone marrow examination can be performed even with less than 10,000 platelet count, which we frequently do in patients with possible ITP or leukemia etc. In fact, no platelet or white cell count is a contraindication for bone marrow examination. Thus, if a patient needs bone marrow examination, it should be performed sooner rather than later, to reach diagnosis early, as many diseases diagnosed by this test, are serious.
Que: What are the major diseases causing pancytopenia?
Ans: Diseases with hypocellular marrow have been discussed in episode 44, under diagnosis of aplastic anemia – mainly aplastic anemia, hypocellular myelodysplastic syndrome, hairy cell leukemia, myelofibrosis, tuberculosis, anorexia nervosa, post radiation or chemotherapy, and others. Hypercellular marrow with pancytopenia is commonly caused by leukemia, myelodysplastic syndrome, other cancers infiltrating marrow, hypersplenism, sepsis, collagen vascular diseases, alcohol, megaloblastic anemia, HIV, brucellosis, and others. List is very long, but available in books and internet. Important thing is to learn how to approach, and make early diagnosis. Some diseases can have either hypo or hyper cellular marrow.
Dr. Chirag A. Shah; M.D. Oncology/Hematology (USA), 079 26754001. Diplomate American Board of Oncology and Hematology. Ahmedabad. firstname.lastname@example.org Shyam Hem-Onc Clinic. 402 Galaxy, Near Shivranjani, Opp Jhansi ki Rani BRTS, Ahmedabad. www.shyamhemoncclinic.com