Myelokathexis is a congenital disorder of the white blood cells that causes severe chronic leucopenia (white blood cell deficiency) and neutropenia (abnormally low level of Neutrophils or White Blood Cells). Though the disease process has not been understood well enough, the condition refers to the retention of abnormal mature neutrophils and bone marrow. This disorder has been inherited in an autosomal dominant manner. Myelokathexis can also signify degenerative changes and hypersegmentation of bone marrow myeloid cells. In simple words myelokathexis refers to a rare congenital neutropenia. This condition arises due to the impaired release of granulocytes from the bone marrow. It is more to do with disorders caused by abnormalities in genes or chromosomes. This condition is usually present from birth.
Symptoms of Myelokathexis
The following symptoms are largely reported in people suffering from myelokathexis
Hyper plastic changes in bone marrow
Shortness of breath
The disease can be diagnosed with blood tests and bone marrow test. Bone marrow transplantation and GM CSF - granulocyte colony stimulating factor and GM CSF - granulocyte macrophage colony stimulating factor are used in treating this condition
Leukopenia is a condition wherein the leukocyte count in the blood is low. Low WBC or White Blood Cells indicate decrease in disease fighting cells circulating in the blood. Some of the common causes for low WBC count:
Viral infections Any viral infections may disrupt bone marrow function for a short duration thus producing low counts of white blood cells. Infections like typhoid, influenza may lower the white blood cell count.
Congenital disorders may weaken bone marrow function, a WBC spectrum test can confirm any such congenital disorder.
Kostmann's syndrome is a congenital disorder wherein the neutrophil production is low.
Myelokathexis Neutrophils fail to enter the blood stream.
Infectious disease HIV destroys the WBC and leaves the person susceptible to infections.
Vitamin or mineral deficiencies
Autoimmune disorders may destroy white blood cells or bone marrow cells.
Aplastic anemia In this condition the bone marrow does not produce enough of any of the cells including white blood cells. This condition may set in all of a sudden or can develop and progress gradually. Few drugs, pregnancy, radiation therapy or chemotherapy can trigger this condition.
Lupus is an auto immune disease wherein the body fights with its own immune system thus destroying white blood cells.
Chemotherapy and radiation therapy are used to kill cancerous cells. They however destroy healthy white blood cells thus leaving the patient with low WBC count. This is one major reason why cancer patients are unable to fight diseases or infections in general.
Leukemia is a type of cancer wherein the body produces too many white blood cells that are abnormal. These white blood cells are not active white blood cells that can fight infections. People down with leukemia have low white blood cell count as their bone marrow is producing more of the abnormal white blood cells.
Cancer may also damage the bone marrow.
Hyperthyroidism An overactive thyroid can produce lower number of white blood cells. The medication used for thyroid can reduce the white blood cell count in the blood.
Liver disorder/Spleen disorder Hypersplenism, in which blood cells are destroyed prematurely by the spleen. An enlarged spleen also known as splenomegaly can trigger low white blood cell count.
Bibliography / Reference
Collection of Pages - Last revised Date: March 23, 2019