Presentation on theme: "Aplastic Anemia Rakesh Biswas"— Presentation transcript:
1Aplastic Anemia Rakesh Biswas MD, Professor, Department of Medicine, People's College of Medical Sciences, Bhanpur, Bhopal, IndiaCase history:A 41 year old lady Extreme pallor, gum bleeds, Purpura, Menorrhagia for one month and fever with mouth ulcers for one week. No organomegalyThis is the link to aplastic anemia patient's story.
2Morphologic, 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.
3Definition:Pancytopenia with hypocellularity (Aplasia) of Bone MarrowOne cell line may be affected more than the others
6PathogenesisImmune mechanism responsible for most of the cases of Idiopathic acquired aplastic anemiaActivated Cytotoxic T cells in Blood & Bone marrow Bone marrow failure
7Clinical Features Signs & symptoms of : Anemia:………. Bleeding: Ecchymoses ,Bleeding gums, EpistaxisInfections: Fever,Mouth ulcersCase 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
8Diagnosis Blood peripheral smear : Pancytopenia and reticulocytopenia Bone marrow aspiration & biopsy : Hypocellular / aplastic bone marrow with increased fat spacesTests for underlying cause ( viral titers)
9Other causes of Pancytopenia: Drugs,Megaloblastic anemiaBone Marrow infiltration or Replacement: Lymphoma, Myeloma,Acute Leukemia, SecondariesHyperspleenisnSLEDisseminated TBPNHSepsis
12BM biopsy hypocellular ,increased fat spaces Hypoplastic marrowCase HistoryMy 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
13Text book Treatment Treatment of underlying cause –if possible Removal of causeSupportive careBlood & platelet transfusionInfection: Broad spectrum antibioticsAsepsisBone Marrow Transplant (SCT)patient age <40yrs , availability of a HLA-identical sibling marrow donor
14Glucocorticoids : in cong Pure Red Cell Aplasia Immunosuppression:Cyclosporine,Glucocorticoids : in cong Pure Red Cell AplasiaAntilymphocyte or Antithymocyte globulin (ALG / ATG)CyclophosphomideAndrogensThymectomy : for Adult Pure Red Cell AplasiaCase 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.
15Case 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
16Hickman catheterCase 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.
17Severe AA (SAA) Bad prognosis Two of three peripheral blood criteria: Neutrophils < 500 / cmm,Platelets < 20,000/cmm,Reticulocyte < 0-0.5%
18Prognosis Improved survival with newer treatment modalities Relates to severityEvolution 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
19Agranulocytosis Leukopenia: Decrease in Total Leukocyte Count Neutropenia: Decrease in Neutrophil count < 1500 / micro LAgranulocytosis: severe neutropenia < 500 neutrophils / micro LAgranulocytosis 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.
21Clinical Features Mouth infection,Sore throat ( Mucositis) Ulcers of : Mouth & throat , Skin, AnusFeatures of Sepsis (Gm +ve &–ve):Fever +/-Hypotension,MODSIn prolonged neutropenia Fungal infections are likely to develop: Candida (Oral), Aspergillus(Pulm)
22Investigations 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.