Aplastic Anemia – Hematopoietic Malignancies and Aplastic Anemia

by Paul Moss, PhD

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    00:02 Finally, let's finish with another important hematological disease, although not a malignant disorder.

    00:10 This is aplastic anemia.

    00:13 And this is a rare condition.

    00:15 But it's characterized by low blood counts due to the fact that there's a reduction in cellularity of the bone marrow.

    00:24 Just look at that slide on the right, that is a bone marrow biopsy, a terrifying biopsy of the bone marrow.

    00:33 And you can see there are too many holes in that, too many fat cells, not enough hemopoiesis.

    00:43 Now, why would this arise? Well, in adults, a probably arises we think, because the immune system is somehow damaging the stem cells within the bone marrow and reducing their numbers.

    00:58 Just to get our terms correct, bone marrow may be actually hypoplastic that means a reduced cellularity or completely aplastic empty.

    01:12 Out of interest, that slide on the right I would call hypoplastic and you can see there is still some hemoptysis there, but it's reduced from what it normally would be.

    01:23 Now then, let's think about why aplastic anemia may arise.

    01:30 Now, occasionally, we see this in children.

    01:34 And this usually arises because of inherited abnormalities that they've got due to damage genes from their mother, or father.

    01:42 Fanconi Anemia, is one of the most common of these collectively rare disorders.

    01:49 And you'll see, on the top right, some of the skeletal abnormalities in the digits that you can see in Fanconi Anemia.

    01:58 And below that some of the otter skin pigmentation that you may also see here.

    02:05 But in adults, aplastic anemia usually arises out of the blue, as we call idiopathic. No obvious reason why.

    02:15 Although it may occasionally follow episodes of hepatitis or an unusual reaction to drug therapy.

    02:24 We make the diagnosis by doing that all important bone marrow trephine, which shows the empty bone marrow lacking in stem cells.

    02:36 Then what can we do about it? Well, treatment has two major components.

    02:43 One is supportive therapy.

    02:45 We keep the patient alive despite the fact that their bone marrow is not working properly.

    02:50 We can transfuse red cells. We can transfuse platelets.

    02:56 Neutrophils are more challenging, and we don't usually do that and we try to control infection with antibiotics and antifungal agents.

    03:07 A specific treatment aims to reverse the process of aplasia and start the bone marrow being produced again.

    03:17 And here we tend to use immune suppression with a drug is called anti-lymphocyte globulin.

    03:25 This is an unusual product in which human lymphocytes are injected into an animal, perhaps a horse, or rabbit even.

    03:34 And those animals will make antibodies against the human lymphocytes, which we can purify and give to patients.

    03:41 And you can imagine that that reduces the lymphocytes in the patient, and it's quite an immune suppressive therapy.

    03:50 It's been proven to be pretty highly effective in patients with aplastic anemia.

    03:56 We often combine this with a drug called cyclosporine.

    04:00 That's an immune suppressant drug as well, and is often used in people who have had a kidney transplant to reduce organ rejection.

    04:10 In patients with moderate disease, that would be the approach you would take if they had a very severe aplastic anemia with very low blood counts.

    04:19 You may also think of a bone marrow transplant an allogeneic bone marrow transplant from another person and that can be highly effective in people with severe disease.

    04:34 So in summary.

    04:36 What we've seen in this lecture is that a wide variety of disorders may develop within the hemopoietic system.

    04:43 Myelodysplasia results from a wide range of mutations that damage the ability of hematopoietic stem cells to produce functional and effective blood cells.

    04:55 Myeloproliferative diseases have a well defined genetic basis and lead to over active bone marrow, often with clinical splenomegaly.

    05:05 And myeloma is a malignant disease of plasma cells leads to bone damage, and paraprotein which itself can cause a range of clinical problems.

    05:15 Finally, aplastic anemia is a rare but serious condition that may result from stem-cell failure perhaps as a result of an inherited abnormality or from autoimmune damage.

    05:30 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. 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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