Blood disorders are diseases that affect the way one or more of the various parts of the blood work in the body. Important components/parts of the blood include red blood cells, which carry oxygen throughout the body to organs and tissues, white blood cells, which are a part of the immune system that help fight infection, platelets, which help form clots in the blood, and plasma, the liquid part of the blood. Blood disorders can cause too much bleeding after an injury or during surgery, childbirth, or a menstrual period, or they can cause blood to clot too easily, which can lead to deep vein thrombosis, stroke, heart attack, or pulmonary embolism (blood clot in the lung). Many blood disorders cause anaemia, a decrease in red blood cells, which can cause weakness and tiredness, paler skin than normal, shortness of breath, dizziness, and fast or irregular heartbeats. Blood disorders can be inherited (genetic, or passed from a family member), or acquired, because of medications, vitamin/nutrient deficiencies, or other health conditions, including certain kinds of cancers. Examples of blood disorders are listed below:
Anaemias Clotting Disorders (aka “Hypercoagulation” or “Thrombophilia”)
-Aplastic Anaemia -Antiphospholipid Syndrome
-Autoimmune Haemolytic Anaemia -Antithrombin Deficiency
-Diamond-Blackfan Anaemia -Disseminated Intravascular Coagulation/DIC
-Fanconi Anaemia -Paroxysmal Nocturnal Haemoglobinuria
-Haemolytic Anaemia -Protein C Deficiency
-Iron-deficiency Anaemia -Protein S Deficiency
-Macrocytic Anaemia -Prothrombin Gene Mutation
-Megaloblastic Anaemia -Factor IV Leiden
-Microcytic Anaemia Bleeding Disorders
-Normocytic Anaemia -Fibrinogen Deficiency
-Pernicious Anaemia -Inherited Haemophilia
-Sickle Cell Anaemia -Type A (“Classic Haemophilia”)
-Sideroblastic Anaemia -Type B (“Christmas Disease”)
-Thalassemia -Type C (“Rosenthal Syndrome”)
Blood Cancers -Thrombocytopenia
-Multiple Myeloma -Immune Thrombocytopenia (ITP)
-Myelodysplastic Syndrome -Idiopathic Thrombocytopenic Purpura (ITP)
-Leukaemia -Thrombotic Thrombocytopenic Purpura (TTP)
-Lymphoma -Von Willebrand Disease
Treatment for blood disorders may include blood transfusions, bone marrow/stem cell transplants, blood donation for disorders of too many blood cells, or removal of the spleen, which stores extra blood, filters blood, and creates some blood cells. Blood disorders related to nutrient deficiencies can often be treated with a change in diet. There are many medications available to treat blood disorders, and the treatment depends on the type of blood disorder.
B Vitamin Supplements treat anaemias related to B-vitamin deficiency. Vitamin B-12/cyanocobalamin and Vitamin B-9/folic acid/folate are necessary for the proper development of red blood cells.
Iron Supplements treat iron-deficiency anaemia, especially when increasing iron in the diet alone is not effective. Iron is needed to form haemoglobin, which is the oxygen-carrying part of red blood cells. Usually, iron is given in the form of oral ferrous sulphate, ferrous gluconate, or multivitamins with iron. In severe iron-deficiency anaemias, iron infusions may be needed. Examples include iron dextran, iron sucrose, iron isomaltoside, and ferric carboxymaltose.
Erythropoietin is a hormone that helps your body make red blood cells. Man-made versions (darbepoetin alfa, epoetin alfa) are given to some anaemic patients.
Corticosteroids suppress the immune system to treat autoimmune causes of anaemia or other immune-related blood disorders (i.e.- ITP). Examples of corticosteroids include prednisone, prednisolone, methylprednisolone, and dexamethasone.
Sickle Cell Anaemia Treatments are discussed in more detail here because this is a significant health concern for many South Africans. Sickle Cell Disease is an inherited blood disorder that caused the red blood cells to be shaped like crescent moons, or “sickles.” This causes them to get stuck in blood vessels, which can cause extreme pain and damage organs and blood vessels.
Clotting Factor Replacement is the mainstay of therapy for people with haemophilia. Concentrated clotting factors either made from human blood plasma or in a lab are given to help control bleeding and to help prevent major bleeding events (prophylaxis).
Birth Control Pills, or oral contraceptives/OCPs, may be given to females with bleeding disorders to decrease the amount of blood lost during menstrual periods. The hormones in OCPs increase clotting factors in the blood.
Vitamin K is an essential vitamin for blood clotting. It increases clotting factors in the clotting cascade. Oral or injected vitamin K/phytonadione may be given as a supplement for clotting disorders caused by vitamin K deficiency, or injected vitamin K may be given in emergencies to control bleeding.
Antifibrinolytic agents prevent blood clots from breaking down, helping blood to clot for longer. These may often be given to patients with bleeding disorders before dental work or surgery, or after childbirth, to help control bleeding. Examples include aminocaproic acid and tranexamic acid.
Desmopressin, also called DDAVP, is a manmade version of a hormone called vasopressin that helps decrease bleeding.
Emicizumab is a medication that imitates a clotting factor (a factor VIII “mimetic”) to assist with blood clotting.
Anticoagulants work on chemicals involved in the clotting cascade (process of clot formation) to decrease the blood’s ability to clot. These can help to treat a clot that has formed, and/or to decrease the likelihood of other clots forming. Oral anticoagulants include warfarin, dabigatran, rivaroxaban, and edoxaban. Warfarin requires careful monitoring with regular bloodwork. Injectable anticoagulants include heparin, enoxaparin, dalteparin, and fondaparinux.
Antiplatelets prevent platelets from sticking together. Examples include aspirin, clopidogrel, cilostazol, and ticagrelor.
Cancers of the blood include abnormally high or low numbers of certain blood cells. Treatment includes radiation therapy, bone marrow/stem cell transplant, and chemotherapy medications.
Chemotherapy drugs work by killing/destroying cancer cells or stopping their growth, or by stimulating the immune system to fight the cancer. Examples of chemotherapy medications used in the treatment of blood cancers include vincristine, daunorubicin, doxorubicin, methotrexate, cyclophosphamide, pegaspargase, 6-mercaptopurine, cytarabine, idarubicin, gemtuzumab, azacytidine, acalabrutinib, alemtuzumab, tretinoin, bendamustine hydrochloride, etoposide, fludarabine, melphalan, teniposide, topotecan. Corticosteroids such as prednisone and dexamethasone are sometimes used along with chemotherapy.
Hematopoietic Growth Factors are substances that help the bone marrow produce more blood cells. These can help regulate blood cell counts that are abnormal either due to chemotherapy or the cancer itself. Epoetin and darbepoetin alfa are red blood cell growth factors. Filgrastim, pegfilgrastim, and sargramostim are white blood cell growth factors. Romiplostim and eltrombopag are platelet growth factors.
Common side effects with many of these medications include nausea/vomiting, diarrhoea, constipation, dizziness, headache, and rash. More serious side effects are often related to the action of drugs.
Bleeding or Blood Clots are possible side effects of medications for clotting disorders or bleeding disorders, respectively, because of how the medications work. If you experience unexplained bruising, excessive nosebleeds, coughing up blood, blood in your urine, stool, or vomit, shortness of breath, severe headache, numbness in arms, legs, or face, chest pain, or pain and warmth in one area of an arm or leg, you should seek medical attention immediately.
Infection Risk is high with some of these medications, especially chemotherapy and corticosteroids. You should practice frequent, correct handwashing, avoid contact with people who are sick, and consider masking to avoid respiratory illness when taking these medications. If you develop signs of infection such as fever, cough, body aches, or shortness of breath, you should seek medical attention right away.
Drug Interactions can be significant and require changes in therapy or affect bleeding risk with some of these medications. It is important that your doctor and/or pharmacist are aware of every medication you are taking, including over-the-counter medications and supplements so that they can monitor for drug interactions.
With the help of medications and other medical treatments, it is possible to live a relatively normal life with a blood disorder. It is important that you take your medications exactly as prescribed, keep all medical appointments, have routine bloodwork as directed, and notify your healthcare provider of any changes in your condition.