Aplastic Anemia – Hematopoietic Malignancies and Aplastic Anemia

by Paul Moss, PhD

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    00:01 Finally, let us finish with another important haematological disease although not a malignant disorder. This is aplastic anaemia. This is a rare condition, but it is characterized by low blood counts due to the fact that there is a reduction in the cellularity of the bone marrow.

    00:24 Just look at that slide on the right. That is a bone marrow biopsy, a trephine biopsy of the bone marrow and you can see there are too many holes in that, too many fat cells not enough haemopoiesis. Now, why would this arise? Well in adults, it probably arises.

    00:50 We think because the immune system is somehow damaging the stem cells within the bone marrow and reducing their numbers. Just to get all terms correct, bone marrow may be actually hypoplastic that means a reduced cellularity or completely aplastic empty. Out of interest that slide on the right, I would call hypoplastic and you can see there is still some haemopoiesis there but is reduced from what normally it would be. Let us think about why aplastic anaemia may arise. Now occasionally we see this in children and this usually arises because of inherited abnormalities that they have got due to damaged genes in their mother or father.

    01:44 Fanconi anaemia is one of the most common of these collectively rare disorders and you will see on the top right some of the skeletal abnormalities in the digits which you can see in Fanconi anaemia and below that some of the. . . skin pigmentation which you may also see here. But in adults, aplastic anaemia usually arises out of the blue and we call idiopathic. No obvious reason why. Although it may occasionally follow episodes of hepatitis or an unusual reaction to drug therapy. We make the diagnosis by doing that all important bone marrow trephine which shows the empty bone marrow lacking in stem cells.

    02:39 Then what can we do about it? The treatment has two major components. One is supportive therapy.

    02:48 We keep the patient alive despite the fact that bone marrow is not working properly.

    02:55 We can transfuse red cells. We can transfuse platelets. Neutrophils are more challenging and we do not usually do that and we tried to control infection with antibodies and antifungal agents.

    03:09 The specific treatment aims to reverse the process of aplasia and start the bone marrow being produced again and here we tend to use immune suppression with the drug as called anti-lymphocyte globulin. This is an unusual product in which human lymphocytes are injected into an animal perhaps the a or rabbit even and those animals will make antibodies against the human lymphocytes, which we can purify and give to patients and you can imagine that reduces the lymphocytes in the patient and is quite an immune suppressive therapy. It has been proven to be pretty highly effective in patients with aplastic anaemia.

    04:01 We often combine this a drug called cyclosporin. That is an immune suppression drug as well and is often used in people who had a kidney transplant to reduce organ rejection. In patients with moderate disease that would be the approach, she would take if they have a very severe aplastic anaemia with very low blood counts. You may also think of a bone marrow transplant and allogeneic bone marrow transplant from another person and that can be highly effective in people with severe disease. So In summary, what we have seen in this lecture is that a wide variety of disorders may develop within the haemopoietic system. Myelodysplasia results from a wide range of mutations that damage the ability of haemopoietic stem cells to produce functional and effective blood cells. The myeloproliferative disease has a well-defined genetic basis and leads to overactive bone marrow often with clinical splenomegaly. A myeloma is a malignant disease of plasma cells leads to bone damage and a paraprotein, which itself can cause a range of clinical problems. Finally, aplastic anaemia is a rare, but serious condition that may result from stem cell failure perhaps as a result of an inherited abnormality or from auto-immune damage.

    05:35 Thanks for watching this lecture on hemopoietic malignancy.

    About the Lecture

    The lecture Aplastic Anemia – Hematopoietic Malignancies and Aplastic Anemia by Paul Moss, PhD is from the course Hematologic Disorders.

    Included Quiz Questions

    1. Hb 14, WBC 9, platelets 700
    2. Hb 8, WBC 8, platelets 1000
    3. Hb 19, WBV 10, platelets 620
    4. Hb 8, WBC 8, platelets 400
    5. Hb 14, WBC 9, platelets 300
    1. Fanconi anemia
    2. Cystic fibrosis
    3. Thalassaemia
    4. Sickle cell anemia
    5. Muscular dystrophy
    1. Bone marrow trephine biopsy
    2. Bone marrow aspiration
    3. Peripheral smear
    4. Genetic analysis
    5. PCR
    1. Plasmapheresis
    2. Cyclosporin
    3. Allogenic stem cell transplant
    4. Anti-lymphocyte globulin
    5. Transfusion of red cells and platelets

    Author of lecture Aplastic Anemia – Hematopoietic Malignancies and Aplastic Anemia

     Paul Moss, PhD

    Paul Moss, PhD

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