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Aplastic Anemia Rakesh Biswas

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1 Aplastic Anemia Rakesh Biswas
MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, India Case history: A 41 year old lady Extreme pallor, gum bleeds, Purpura, Menorrhagia for one month and fever with mouth ulcers for one week. No organomegaly This is the link to aplastic anemia patient's story.

2 Morphologic, Etiologic Possible causes: Investigations and treatment
Case history: In a patient’s own words: I had gone to the emergency room after fainting. I had an extremely heavy period, a terrible headache, a bleeding sinus infection, a gash from falling onto my glasses, painful mouth sores, bruises from where my cat jumped on my lap, red spots all over, and no energy.

3 Definition: Pancytopenia with hypocellularity (Aplasia) of Bone Marrow One cell line may be affected more than the others


5 Etiology Inherited Fanconi’s Anemia Acquired
Idiopathic (majority)-2/3rd of cases Drug : Acetazolamide, Carbamazepine, Gold, Hydantoin, Penicillin, Phenylbutazone, Chemical Radiation exposure Viral illness

6 Pathogenesis Immune mechanism responsible for most of the cases of Idiopathic acquired aplastic anemia Activated Cytotoxic T cells in Blood & Bone marrow  Bone marrow failure

7 Clinical Features Signs & symptoms of : Anemia:……….
Bleeding: Ecchymoses ,Bleeding gums, Epistaxis Infections: Fever,Mouth ulcers Case history: Initial Counts: Hemoglobin: 4.7 WBC: 900 GNC: 23 (not 2300) Platelet: 8,400 My local hospital did a Bone Marrow Biopsy to determine if it was Aplastic Anemia. Within minutes of receiving the results, arrangements were made to transfer me to a higher centre

8 Diagnosis Blood peripheral smear : Pancytopenia and reticulocytopenia
Bone marrow aspiration & biopsy : Hypocellular / aplastic bone marrow with increased fat spaces Tests for underlying cause ( viral titers)

9 Other causes of Pancytopenia:
Drugs, Megaloblastic anemia Bone Marrow infiltration or Replacement: Lymphoma, Myeloma,Acute Leukemia, Secondaries Hyperspleenisn SLE Disseminated TB PNH Sepsis

10 BM Aspiration BM Biopsy

11 Normal marrow

12 BM biopsy hypocellular ,increased fat spaces
Hypoplastic marrow Case History My oncologists explained Aplastic Anemia, and my treatment options. A bone marrow transplant was one option, but even with a related donor match, at age 41, my odds of survival were quoted at around 60%. A third option that they presented was High Dose Cyclophosphamide which was experimental (the same chemotherapy that they use before a bone marrow transplant), without the Bone Marrow Transplant. Immunosuppressent treatment (ATG) was another option. BM biopsy hypocellular ,increased fat spaces

13 Text book Treatment Treatment of underlying cause –if possible
Removal of cause Supportive care Blood & platelet transfusion Infection: Broad spectrum antibiotics Asepsis Bone Marrow Transplant (SCT) patient age <40yrs , availability of a HLA-identical sibling marrow donor

14 Glucocorticoids : in cong Pure Red Cell Aplasia
Immunosuppression: Cyclosporine, Glucocorticoids : in cong Pure Red Cell Aplasia Antilymphocyte or Antithymocyte globulin (ALG / ATG) Cyclophosphomide Androgens Thymectomy : for Adult Pure Red Cell Aplasia Case history: I did not want to spend a lifetime tackling the graft-vs.-host problems associated with a Bone Marrow Transplant if I didn't have to. I also did not want to have the possiblity of a relapse 10 years down the line. The high dose Cyclophosphamide did not appear to cause either of these. The disadvantages of the Cyclophosphamide were the long, slow, vulnerable recovery period with low white counts, and the small but real chance of a fatal reaction to the chemotherapy. I chose the cyclophosphamide. The Cyclophosphamide's job, as I understand it, is to kill off the white blood cells, as they are malfunctioning, and let new ones grow from the stem cells, which Cyclophosphamide does not damage. Kind of a chemical "rebooting" of the blood.

15 Case History: My first post-Cyclophosphamide white cells appeared 10 days after treatment. I had 6. I ordered them all little party hats, and got to know them personally:-) Case history: A month after treatment, I had 160 of those little rascals and 11 neutrophils. At one month, 10 days, I had 600 white cells and 420 neutrophils, and they let me go back home, reporting to my local physician twice weekly, and still on antibiotics

16 Hickman catheter Case History: I took care of my pal, Hickman, with daily Heparin flushes, and twice weekly dressing changes. Dressings were changed with gloves, mask, and betadine swabs. When it was time for us to part company, he was simply tugged out during outpatient surgery.

17 Severe AA (SAA) Bad prognosis Two of three peripheral blood criteria:
Neutrophils < 500 / cmm, Platelets < 20,000/cmm, Reticulocyte < 0-0.5%

18 Prognosis Improved survival with newer treatment modalities
Relates to severity Evolution to MDS, PNH, AML` Case history: My counts have risen slowly but steadily since treatment. I have had no long term physical effects . I have my life back! And it is GOOD. I appreciate it so much more now, and am living it more carefully. Recent UPDATE: Some counts still rising. Hb: 13.3 WBC: 3800 Plt: 165k GNC: 2100 Hct: 38.6

19 Agranulocytosis Leukopenia: Decrease in Total Leukocyte Count
Neutropenia: Decrease in Neutrophil count < 1500 / micro L Agranulocytosis: severe neutropenia < 500 neutrophils / micro L Agranulocytosis should be differentiated from other syndromes of bone-marrow failure, including pancytopenia and aplastic anemia. Leukemia should be excluded. The basic difference from aplastic is here only the myeloid series is affected.

20 Causes Congenital Drug induced:Chloramphenicol,CBZ, Carbimazole , Co-trimoxazole, Gold, Phenytoin, Sulfa drugs Infections: Viral-Hepatitis,Influenza,HIV Bacterial-Typhoid,Miliary TB Benign ( familial/racial) Cyclical Immune: AI, SLE,Felty’s,

21 Clinical Features Mouth infection,Sore throat ( Mucositis)
Ulcers of : Mouth & throat , Skin, Anus Features of Sepsis (Gm +ve &–ve): Fever +/- Hypotension, MODS In prolonged neutropenia Fungal infections are likely to develop: Candida (Oral), Aspergillus(Pulm)

22 Investigations and Treatment
The peripheral blood smear shows a marked decrease or absence of neutrophils. The bone marrow may show myeloid hypoplasia or absence of myeloid precursors. In many cases, the bone marrow is cellular with a maturation arrest at the promyelocyte stage. On occasion, the marrow may be hypercellular.

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