G-CSF allows patients to: better tolerate chemotherapy and decreased febrile neutropenic infections The meta-analysis could not determine with statistical significance whether patients treated with G-CSF lived longer or remained cancer-free for longer than those in the control group. In the end, this may also impact the: length of hospital stay. The fact that G-CSF helps [...]
Hematology
The main syndrome of vitamin B12 deficiency is Biermer’s disease (pernicious anemia). It is characterized by a triad of symptoms: Anemia (megaloblastic anemia) Gastrointestinal symptoms Neurological symptoms Each of those symptoms can occur either alone or along with others. The neurological complex, defined as myelosis funicularis, consists of the following symptoms: Impaired perception of deep [...]
von Willebrand’s Disease is a mild manageable bleeding disorder in which clinically severe hemorrhage manifests only in the face of trauma or invasive procedures. The most common symptoms include nosebleeds, skin bruises, and hematomas. Prolonged bleeding from trivial wounds, oral cavity bleeding, and excessive menstrual bleeding are common. Gastrointestinal bleeding is rare. A common but nonspecific [...]
Autosomal recessive male/female equal Other terms for this disorder include plasma thromboplastin antecedent (PTA) deficiency, Rosenthal syndrome, and hemophilia C. Racial predilection for people of Jewish heritage. Look for this! An unexpected and incidental preoperative finding of a prolonged activated partial thromboplastin time (aPTT) and no explanation may prevent a scheduled surgery. In contrast to hemophilias [...]
Due to inappropriate increase in iron resorption from intestine or from iron overload due to multiple transfusions. Symptoms include skin pigmentation (90%), diabetes mellitus (65%), liver impairment (hepatomegaly in 95%), cardiac impairment (CHF in 10-15%), arthropathy (25-50%), and hypogonadism. Treatment involves phlebotomy (weekly or biweekly – 500 mL initially, later one every 3 months), and [...]
Paroxysmal nocturnal hemoglobinuria is an acquired clonal disease in which some red blood cells have no way to attach proteins to outer membrane (proportion of abnormal cells varies from patient to patient). Paroxysmal nocturnal hemoglobinuria (PNH) is a descriptive term for the clinical manifestation of red blood cell (RBC) breakdown with release of hemoglobin into [...]
Most common defect in the hexose-monophosphate shunt Probably partially protects from malaria infection Affects about 200 million people worldwide Patient may experience a hemolytic crisis within hours of exposure to oxidant stresses including: – Antimalarials (primaquine, pamaquine, dapsone), – Sulphonamides (sulfamethoxazole), – Nitrofurantoin, – Analgesics (acetanilid), – Miscellaneous (Vit.K, doxorubicin, nalidixic acid, methilene blue, niridazote [...]
Normochromic, normocytic anemia Low serum iron levels Low transferrin saturation Low or normal serum transferrin levels High serum ferritin levels Hence, abundant iron storage yet transfer to the RBC is impaired.
Vitamin B 12 deficiency Folate deficiency Liver disease Hemolysis Alcohol abuse Bone marrow abnormalities Drug effect (hydroxyurea, etc.)
Defined as mean corpuscular volume (MCV) < 82 fl Typical causes of microcytic anemia include: childhood iron deficiency anemia, by far the most common cause of anemia in general and of microcytic anemia in particular thalassemia adulthood iron deficiency anemia sideroblastic anemia, congenital or acquired sometimes, anemia of chronic disease, although this more typically causes [...]
Overproduction of erythrocytes, platelets and granulocytes Most common of chronic myeloproliferative disorders Criteria for the diagnosis of the polycytemia vera: – elevated red cell mass, – normal arterial oxygen saturation, – splenomegaly, – in the absence of splenomegaly : Leukocytosis and thrombocytosis AND, – plasma erythropoetin level less than 4 mU/mL. These criteria are not [...]
The cause of thrombotic thromocytopenic purpura is unknown. It manifests as arteriolar lesions in various organs that contain platelets and fibrin. This results in thrombocytopenia and hemolytic anemia due to fragmentation of red cells. Tissue hypoxia causes organ dysfunctions. CNS and kidneys are most markedly involved. Mostly affects young women The pentad of findings associated [...]
Hemophilia B is a hereditary bleeding disorder caused by a lack of blood clotting factor IX. Without enough factor IX, the blood cannot clot properly to control bleeding. Also known as Factor 9 deficiency, or “Christmas Disease” Normal PT and increased PTT Symptoms include easy bruising, muscle, and joint hemorrhages. X-linked recessive transmission, and the less [...]
Factor 8 deficiency Normal PT and increased PTT Symptoms include easy bruising, muscle, and joint hemorrhages. X-linked recessive For mild disease treat with DDAVP. For severe disease use FFP, cryoprecipitate, or factor 8 concentrate.
This is a disorder characterized by spherocytosis, increased RBC fragility, and extra-vascular hemolysis. Clinical Picture: mild hemolytic anemia, jaundice, gallstones, and splenomegaly. Aplastic crisis can occur with parvovirus infection. Severe cases can have dermatitis with ulcerations. Diagnose with direct blood smear visualization. Labs: High unconjugated bili, low haptoglobin, reticulocytosis. Osmotic hemolysis test demonstrates increased RBC [...]