The problem with Sickle Cell is that it’s a bit too complicated and most people don’t understand how it works. So here are a few basic facts from you might want to know.
[We’re not doctors or medical professionals. We recommend you seek professional medical advice if you think you may be affected by Sickle Cell. If you have any more suggestions to add to this list please let us know]
1. You can’t “catch” Sickle Cell
Sickle Cell Disease (also known as Sickle Cell Disorder or Anaemia) is not contagious. Repeat, you can not catch it. It’s passed down to a child from its parents genetically.
2. Sickle Cell is NOT a terminal disease
Sickle Cell is very painful and serious disease which can cause a range of health issues and in some cases short life expectancy. In Nigeria 98% of children born with Sickle Cell Disease die before they’re 5 years old. However, when diagnosed early and with support, it’s also a manageable condition. There’s no practical cure yet, but people with the disorder can live into their 50s, 60s and beyond when they manage their condition carefully. The point is, people with Sickle Cell should be hopeful and their families and friends shouldn’t consign them to an early death.
3. People with Sickle Cell trait are usually perfectly healthy carriers
People who have the genetic trait also known as “AS”, “AC” or one of the Thalassemia variant statuses, do not normally suffer any symptoms. There are some exceptions to this but it’s generally not a health issue if you carry the trait. People who have “full” Sickle Cell Disease, also referred to as SS or SC, will likely have significant health difficulties however.
4. The Sickle Cell “trait” is extremely common in Ghana and other African countries.
Some statistics say that between 20-25% of all Ghanaians and Nigerians have either the Sickle Cell trait AS or AC. Most of us don’t know we have it because it’s normally harmless, so you’d never be able to guess without a proper test. Some of those tests don’t look for AC, so people should specifically ask for AS, AC and Thalassemia screening.
5. If two parents who have the Sickle Cell “Trait” have a baby, it might be born with full Sickle Cell Disease
If a mother and father have one of the Sickle Cell traits, the future child is at significant risk of being born with “full” Sickle Cell Disease. There’s a 1 in 4 chance this will happen. If you choose to take that risk, you should be prepared and speak to a doctor about treatment and testing of the child. Before having children, we recommend you ask a doctor or clinic to check your status and for them to provide genetic counselling if you carry the trait.
6. Getting tested for Sickle Cell trait will NOT ruin your life.
If you have it, the trait doesn’t normally cause any health problems. Then you and your future partner can make informed decisions when it comes to making babies. Remember that if both parents are carriers, there is a 1 in 4 chance the baby will have full Sickle Cell Disease. There are other situations like when one parent has full ‘SS’ sickle cell and the other has ‘AS’. In those cases it’s always wise to talk to an informed, up to date doctor before having children.
7. People with Sickle Cell are not sick all the time!
People with Sickle Cell Disease feel okay most of the time. When they have a ‘crisis’, that’s when the mis-shaped blood cells prevent enough oxygen getting around the body and the pain starts. At this stage, they often need to be admitted to hospital for treatment with pain killers, hydration and other treatments.
8. People with Sickle Cell can live full, productive lives.
Many successful Ghanaians in business, entertainment and government have Sickle Cell Disease. Yes, even though Sickle Cell Disease is a serious health problem, when it’s managed well, sufferers can live productive lives, have children and work. They deserve consideration from others when they’re sick, but they shouldn’t be discriminated against.
9. Don’t wait, don’t leave it too late, find out if you have the trait!
Relax, having the trait is NOT like finding out you have a disease because there are is no suffering and it’s not contagious. You will be simply be able to make informed decisions about having kids with your future or current partner. Go to your doctor, clinic or ask someone you trust to suggest a testing facility near to home.
10. Sickle Cell children can ONLY be born to parents who BOTH carry the trait
That’s right, if a kid has Sickle Cell Disease, the kid’s parents both have the trait, or have Sickle Cell. There are no known exceptions, despite what some men might like to believe.
11. There is no available cure for Sickle Cell Disease
There are medical treatments like Hydroxyurea but no practically available cure. Even though this is a such huge health, social and economic problem in Africa, the research and funding levels are astonishingly low. Maybe that’s because most of the people who have the condition can’t afford to pay for expensive treatments.
12. Sickle Cell Stigma is wrong.
People with Sickle Cell Disease have a tough time, they deserve admiration and support. They didn’t catch it, it happened to them before they were born. We all have friends and family with Sickle Cell Disease, let’s treat them with the respect they deserve.
13. Sickle Cell is not a “curse”
It’s a genetic disorder not a curse. It’s serious, but people who manage their Sickle Cell Disease can live long happy lives. If anyone tries to tell you something different, they don’t know what they’re talking about.
14. People with Sickle Cell are not immune to Malaria
People with the “AS” trait have more immunity than those without, but people with full Sickle Cell Disease have no immunity and are actually at greater risk.
15. Not only black people can have Sickle Cell
Sickle Cell is very common in Africa and amongst people of African descent. However, it’s also prevalent in the Middle East, Asia, North and Latin America.
I prep for sickle cell exchanges all the time in the Blood Bank. They often require specific antigen negative units (often Rh family antigens) or even washing of the unit on top of testing for Hgb S. Some exchanges even exceed 14 units for one transfusion sitting.
Iron-deficiency anaemia is caused by blood loss, insufficient dietary intake, or poor absorption of iron from food.
Iron deficiency anaemia can be prevented by eating a diet containing sufficient amounts of iron or by iron supplementation. Severe cases may be treated with blood transfusions or iron injections.
Cause
Increased iron demand - occurs during periods of growth, such as in children and pregnant women - more iron is used than is taken in by diet
Menstrual bleeding - Women with menorrhagia (heavy menstrual periods) loose blood and therefore iron.
Gastrointestinal bleeding - many sources of gastrointestinal tract bleeding resulting from medication or many different GI pathologies.
Diet - In many countries, wheat flour is fortified with iron. If a person consumes too little iron, or iron that is poorly absorbed (non-heme iron), they can become iron deficient over time. For children, a high intake of cow’s milk is associated with an increased risk of iron-deficiency anaemia.
Iron malabsorption - Iron from food is absorbed into the bloodstream in the small intestine, primarily in the duodenum. Many gastrointestinal disorders can reduce the body’s ability to absorb iron. EG in coeliac disease, abnormal changes in the structure of the duodenum can occur.
Quick note on iron supplements: iron is water-soluble, so an important note advertised in transfusion centers is to take the supplement with vitamin C, rather than water, so it’s actually absorbed (otherwise you will pee it out)!
When it comes to cell identification during a manual differential, it can be hard differentiating reactive (atypical) lymphocytes and monocytes.
The cell on the left is a reactive lymph that the experienced may not confuse with a monocyte. It has a round to oval nucleus with a fine chromatin pattern and a large, basophilic cytoplasm.
The middle cell, however, looks very similar to a monocyte, and by itself, without an actual monocyte for comparison in the same field, may trick some. It has an indented nucleus with a fine/lacy chromatin pattern, just like many typical monocytes do. The cytoplasm is a little less basophilic compared to the left cell and, if you stare at it long enough, can even have the sort of “ground glass” look that is very characteristic of a monocyte’s cytoplasm.
The cell on the right is a very typical monocyte. It has an oddly-shaped nucleus with a lacy chromatin pattern and a blue-gray, ground glass cytoplasm, as well as vacuoles.
For this particular patient, I knew that the cell on the right was a monocyte and not a reactive lymph because its cytoplasm was blue-gray and vacuolated. No other reactive lymph had these features. They all had more basophilic cytoplasms, especially around the edge of the cell and no vacuoles were present. But here’s the problem - reactive lymphocytes can be vacuolated and the cytoplasm of a monocyte can sometimes be more basophilic than blue-gray. It just wasn’t the case for THIS PATIENT. So what do you do if your patient has reactive lymphs and monocytes with similar cytoplasms and both are vacuolated? Try to find similarities between them to categorize them as two separate populations as I’ve done. For me, it was a basophilic cytoplasm and no vacuoles v.s a blue-gray cytoplasm with vacuoles. For another patient, it may be large sized cells with large azurophilic granules (reactive lymphs) vs. medium sized cells and no azurophilic granules (monocytes).
Simply look at the details to see if one population has something the other population that is very similar does not. Reactive lymphs and monos can look nearly identical at times, but sometimes you have to forget what you know and simply look at what you see at first, and then go from there.
When it comes to cell identification during a manual differential, it can be hard differentiating reactive (atypical) lymphocytes and monocytes.
The cell on the left is a reactive lymph that the experienced may not confuse with a monocyte. It has a round to oval nucleus with a fine chromatin pattern and a large, basophilic cytoplasm.
The middle cell, however, looks very similar to a monocyte, and by itself, without an actual monocyte for comparison in the same field, may trick some. It has an indented nucleus with a fine/lacy chromatin pattern, just like many typical monocytes do. The cytoplasm is a little less basophilic compared to the left cell and, if you stare at it long enough, can even have the sort of “ground glass” look that is very characteristic of a monocyte’s cytoplasm.
The cell on the right is a very typical monocyte. It has an oddly-shaped nucleus with a lacy chromatin pattern and a blue-gray, ground glass cytoplasm, as well as vacuoles.
For this particular patient, I knew that the cell on the right was a monocyte and not a reactive lymph because its cytoplasm was blue-gray and vacuolated. No other reactive lymph had these features. They all had more basophilic cytoplasms, especially around the edge of the cell and no vacuoles were present. But here’s the problem - reactive lymphocytes can be vacuolated and the cytoplasm of a monocyte can sometimes be more basophilic than blue-gray. It just wasn’t the case for THIS PATIENT. So what do you do if your patient has reactive lymphs and monocytes with similar cytoplasms and both are vacuolated? Try to find similarities between them to categorize them as two separate populations as I’ve done. For me, it was a basophilic cytoplasm and no vacuoles v.s a blue-gray cytoplasm with vacuoles. For another patient, it may be large sized cells with large azurophilic granules (reactive lymphs) vs. medium sized cells and no azurophilic granules (monocytes).
Simply look at the details to see if one population has something the other population that is very similar does not. Reactive lymphs and monos can look nearly identical at times, but sometimes you have to forget what you know and simply look at what you see at first, and then go from there.
Well it’s called myleogenous leukemia. So obviously the leukocytes will be increased. But, what are myelocytes? They’re baby granulocytic cells, granulocytic cells create basophils and eosinophils. So look for increased white blood cells, granulocytes, and basophils.
Break it down and you can figure out what you’re looking for!
Blowing the cover of the stealthy malaria parasite:
Part of the reason why malaria is so persistent after infection occurs is that it is concealed from detection by white blood cells. Through most of their life cycles, the Plasmodium parasites that cause the disease hide within red blood and liver cells where they are effectively invisible.
That’s where the recently-studied and catchily-named compound (+)-SJ733 comes in. Research has shown this chemical to inhibit one of the parasite’s vital proteins in order to kickstart a series of physical changes for red blood cell in which it resides. This makes the infected cells look aged to the immune system, marking them for retirement by destruction as is normal.
By this mechanism, infected cells are systematically wiped out before the parasite can spread, and normal red blood cells are left unharmed! Trials so far have shown promising results and better yet, there is potential to “develop an affordable, fast-acting combination therapy that cures malaria with a single dose,” according to one of the co-authors of the study. Certainly, an affordable treatment would be welcome in countries where malaria is most prevalent.
Planning has now begun for human trials of the compound. The chances are that it won’t be seen in action for some time to come.
Image: two malaria-infected red blood cells, credit to Lennart Nilsson.
A section of tissue is taken as it would for Histology (see prev post)
The section is then treated with an antibody that recognises the marker we’re looking for. This is referred to as the primary antibody.
The primary antibody is then recognised by a secondary antibody that is linked to an enzyme, or several copies of the enzymes (conjugates).
Finally, the section is treated with a chromogen, a reagent that is acted upon by the enzyme, to deposit an insoluble coloured compound onto the cell, where the original primary antibody had bound.
A molecule that binds to, and is recognised by, an antibody, is called an antigen,
In histology, antigens = markers, since they act as ways of recognising a particular cell.
Many markers are designated by a CD number.
EG different classes of lymphocyte appear virtually identical in size and shape (morphology), but they can be distinguished according to their surface markers.
All T lymphocytes express CD3,
Helper T cells and cytotoxic T cells express CD4 and CD8, respectively.
Therefore, a T cell lymphoma can be tracked in different tissues using these markers.
Double immunolabelling of an islet of Langerhans in the pancreas identifies Insulin-producing cells (blue) and glucagon-producing cells (brown)
Another use for IHC is in guiding treatment.
For example, many breast cancers require oestrogen to divide, but a drug called tamoxifen can bind to the oestrogen receptor on the cancerous cells, blocking this proliferative effect.
Looking at tissue sections to see their normal histology in my new pathology module. Next week we’re looking at cancerous tissues to see the difference. 🔬🦠
