The Different Clinical Manifestations Of Hemolytic Anemias
The Different Manifestations
In addition to the general symptomatology of anemia, hemolysis leads to distinctive features which differ from each other in the acute and chronic forms. Chronic hemolysis leads to jaundice, hemolytic facies, chronic leg ulcers, splenomegaly, cholelithiasis, cutaneous pigmentation and Iron overload states. On the other hand, acute hemolytic episodes are characterized by sharp rise in temperature, marked prostration, hemoglobinuria, acute renal failure, disseminated intravascular coagulation and shock. In many cases, the hemolysis is secondary to other underlying conditions such as lymphomas, leukemia, malignancy or dyscollagenosis.
The different manifestations
Jaundice: Hemolytic jaundice is generally mild but at times this can be severe. The degree of jaundice and the extent of hemolysis may not always correlate.
The facies: It is characteristic in hemolytic anemias. There is enlargement of the skull with frontal and parietal bossing. The malar bones are prominent with flattening of the bridge of the nose, widely set eyes and malocclusion to the teeth. The appearance may be mongoloid. This is seen most in thalassemia major. All these changes are caused by enlargement of the marrow tissue.
Chronic leg ulcers: Chronic non-healing ulcers develop the malleoli in severe hemolytic states such as sickle cell anemia and hereditary spherocytosis. These heal when the anemia improves.
Splenomegaly: Splenomegaly is invariably seen in all cases of hemolytic anemia though the size of the spleen may be variable. Massive splenomegaly occurs in thalssemia and this may lead to hypersplenism.
Cholelithiasis: Pigment stones in the biliary tree are occasional complications seen in chronic hemolysis, caused by the excessive presence of bilirubin in bile.
Crisis: The course of chronic hemolytic anemias may be punctuated by acute episodes called crisis. These are aplastic, megaloblastic, hemolytic, sickling and sequestration crisis. Aplastic crisis is due to sudden marrow failure precipitated generally by viral infection. Megaloblastic crisis is produced by folate deficiency resulting from the excessive demand brough about by accelerated erythropoiesis. Hemolytic crisis is rate, which may develop spontaneously or may be an adverse reaction to drugs or viral infections. Sickling crisis occurs in sickle cell anemia when exposed to hypoxic environment. This leads to vascular occlusion of many organs such as lungs, spleen, kidney and brain. In sequestration crisis, sudden splenomegaly develops because of pooling of blood within the organ and severe anemia ensues rapidly.
Melasma (darkening of the skin)
Darkening of skin and other Iron overload syndromes: Chronic hemolytic anemias lead to Iron overload due to liberation of excessive Iron which is taken up by the macrophages or deposited in the parenchymal cells of several organs. The absorption of Iron from the G.I tract is also increased in thalassemias. Repeated blood transfusion adds to Iron overload. These result in hemosiderosis characterized by grayish complexion of the skin, cirrhosis of liver, diabetes mellitus and cardiomyopathy.
© 2014 Funom Theophilus Makama