The term Apheresis is Greek in origin and it means 'to take away'. This is a medical technology wherein the blood of a donor/patient is passed through an apparatus that separates one particular required component. The separated portion is withdrawn and the remaining components are retransfused into the patient/donor. The components thus separated from the patient/donor includes, plasma, platelets or leukocytes. This procedure is adhered to as an extracorporeal therapy.
In the process of apheresis, whole blood is removed from the patient/donor. The components of whole blood are separated by a centrifuge or an instrument specially designed for this purpose. The apheresis procedure is often employed to obtain stem cells from peripheral blood of patients suffering leukemia, a blood disease. Stem cells are infused into the patient's blood stream in order to produce cells that eventually will mature into red blood cells, white blood cells and platelets.
In transplants such as bone marrow procedures, the sick person happens to be his/her own donor. Blood is drawn and at a later time reinfused into that patient's blood stream in a procedure known as autologous bone marrow transplant. When stem cells are obtained from a healthy person, the procedure is allogeneic bone marrow transplant. Here, another person, may or may not be related, is the donor. Apheresis has become essential in providing blood components for several types of therapies. The process of apheresis takes a couple of hours and the volunteer donor/patient undergoes apheresis to supply specific components.
Leukapheresis is the removal of PMNS (Polymorphonuclear leukocytes), basophils and eosinophils for transfusion into patients whose PMNs are ineffective or in whom the traditional line of therapy has failed. LDL apheresis is the removal of low density lipoprotein in patients suffering from hypercholesterolemia. Erythrocytapheresis is the collection of red blood cells (RBCs) either two standard units of RBCs or one unit plus either plasma or platelets. This process is commonly known as 'double reds' or 'double red cell apheresis'.
Apheresis is used in stem cell harvesting for circulating bone marrow cells during transplantations. To collect sufficient stem cells, apheresis process is performed for at least two consecutive days, although at times five or even more procedures become necessary.
It is pertinent to understand that when apheresis system is adopted for therapy, the system is removing relatively small amounts of fluid, not more than 10.5 mL/kg body weight. That fluid which is removed must be replaced in order to maintain the intravascular volume.
The apheresis process is painless. Some patients/donors may experience lightheadedness, numbness or tingling of the nose, lips or fingers. These symptoms are short lived and treatable. Possible complications include bleeding in needle sites, thrombosis or clotting in blood vessels, or rarely surgical complications if a temporary apheresis catheter is inserted. Infection is always a risk as this procedure involves penetrating the skin and open access to blood vessels.
Myelodysplastic syndrome or MDS refers to a group of blood disorders caused by defective blood cell production in the bone marrow. Bone marrow produces immature blood cells called blasts, which over a period of time develop into mature blood cells and divide themselves into red blood cells, white blood cells and platelets. In Myelodysplastic syndrome, these blasts fail to mature and either die in the marrow itself or soon after they reach the blood stream. When there are not enough healthy blood cells, the body becomes weak and is susceptible to infections. MDS is not a cancer, however, in 20 to 30% of patients, the condition may progress itself into cancer and thus this condition was earlier called preleukemia.
Causes of Myelodysplastic syndrome
Based on causes, Myelodysplastic syndrome can be classified into primary MDS and secondary MDS. Myelodysplastic syndrome presenting itself without any known cause is called primary MDS. Myelodysplastic syndrome may also occur due to some known reasons such as history of cancer treatment involving radiation and chemotherapy, exposure to certain industrial chemicals and smoking. When the cause of the MDS condition is known, it is called secondary Myelodysplastic syndrome. Identifying the type of MDS is vital to the treatment as primary MDS has better prognosis when compared to secondary MDS.
Myelodysplastic syndrome does not cause any symptoms in the initial stages of the disease. However, the following warning signs may show up as the disease starts to progress.
Diagnosis and treatment
MDS is diagnosed with the help of blood tests and bone marrow tests. A complete blood test is done to understand the different blood counts. However, blood tests alone cannot detect MDS. Bone marrow tests are conducted to confirm the presence of Myelodysplastic syndrome. This procedure involves taking bone marrow samples from the pelvic bone of the patient by inserting a needle under local anesthesia. Once MDS is determined, the following methods are followed to treat the condition.
A bone marrow disorder that is mostly inherited, Kostmann's syndrome indicates a severe deficiency in neutrophils. Neutrophil is a type of white blood cell that helps the body fight infections. This condition is referred to as an autosomal recessive disorder of neutrophil production. It is also commonly known as severe congenital neutropenia. This condition leads to recurrent infections right from infancy as this condition is usually diagnosed just after the birth of a baby. Infection of the lungs, sinus glands and liver are common. There are several forms of neutropenia, of which Kostmann's syndrome or congenital neutropenia is common in kids.
People affected with this condition may develop fever and inflammation of the skin and gums. About 40% of the people affected by this disease have decreased bone density thus making the bones brittle. About 20% of the people with this condition are prone to develop leukemia or myelodysplastic syndrome during adolescence. Seizures, heart and genital abnormalities may also be noticed in such patients. If not treated rightly, the condition could prove fatal. The syndrome is named after the Swedish doctor Kostmann who discovered it.
Kids suffering from this condition inherit an abnormal gene from each of their parent. Carriers of the gene do not experience any symptom and so until the child is born the parent wouldn't know that they carried such a gene.
Symptoms of Kostmann's syndrome
Severe neutrophil deficiency
Necrotic ulceration of genital mucosa
Necrotic ulceration of oral mucosa
The disease can be diagnosed with blood tests, genetic testing and bone marrow tests. The physician might administer a substance called granulocyte colony stimulating factor (GCSF). This stimulates the bone marrow to produce more neutrophil. Other forms of treatment include Haematopoietic stem cell transplantation (HSCT), bone marrow transplant and antibiotics.
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Bibliography / Reference
Collection of Pages - Last revised Date: January 28, 2020