HemOnc Anemias

Created by wie1473 

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RBC maturation in Blood

reticulocyte and erythrocyte

RBC Studies

RBC count, Hemoglobin, Hematocrit (packed cell volume)

RBC indices

MCV, MCH, MCHC

Reticulocyte count

indication of hematopoetic/bone marrow activity, norm range 0.5-1.5%

RDW

RBC distribution width, measurement variation in size of RBCs, normal 11-14%, Increased in Vit B12 deficiency, folate deficiency, IDA, Normal in acute blood loss

Basophilic stippling

lead poisoning

T/F anemia is a disease of blood delivery to O2

False. Not a disease or diagnosis in itself but a manifestation of underlying disease

Anemia problem

A decrease in ability of blood to deliver O2 to peripheral tissues resulting in tissue hypoxia

Adequate RBC production requirements

iron, vit B12, and folic acid; hemoglobin synthesis must also function normally

Causes of anemia

Acute bleeding, decrease production of RBCs, increased destruction of RBCs

Normocytic, normochromic anemia

anemia of chronic dz, acute blood loss

Microcytic, hypochromic

Iron deficiency anemia, thalassemias, sickle cell, chronic blood loss

Macrocytic (megaloblastic) anemias

Vit B12 deficiency/pernicious anemia, Folate deficiency, chemotherapy, liver dz

Most common anemia in US

Iron deficiency anemia

Iron absorption

In duodenum and proximal jejunum, enters bloodstream and binds to transferrin, taken to bone marrow to be incorporated into hgb, Stored in liver as ferritin and hemosiderin

Iron Deficiency anemia definition

A decreased total body iron content

Etiologies of IDA

Bleeding, dietary, malabsorption, sources of loss include GI, gyne, urinary tract, lung & skin

IDA clinical findings

fatigue, pallor or mucus membranes, glossitis, angular stomatitis, atrophic gastritis, spooning of nails, pica in severe disease

Lab studies in IDA

Decreased H/H, MCV and MCHC, iron and ferritin levels, transferrin saturation, reticulocytes early, increased TIBC

Most sensitive way to diagnose IDA

Serum ferritin level more sensitive and reliable way to diagnose IDA than serum Fe and TIBC

Treatment of IDA

Iron replacement FeSO4 325mg PO TID or IV, IM. Usually need to continue tx 3-6months after improvement to replete Iron stores and prevent relapse

Pernicious anemia

A megaloblastic anemia from low vit B12 levels (cyanocobalamin), Intrinsic factor is necessary to absorb B12, autoimmune disease due to abs directed against parietal cells or intrinsic factor

Other etiologies of Vit B12 Deficiency

dietary deficiency, gastrectomy, pancreatic insufficiency, Zollinger-Ellison dz, Bacterial overgrowth in intestine, Fish tapeworm, blind loop syndrome

Pernicious anemia Findings

Fatigue, pallor, anorexia, smooth/beefy red/sore tongue, diarrhea or constipation; neuro- paresthesias and balance problems. Severely low B12 levels lead to dementia and neuropsychiatric disturbances

Pernicious anemia lab studies

Decreased H/H, increased MCV, Howell-Jolly bodies in RBCs, may see hypersegmented neutrophils on smear, low Vit B12 level, parietal cell or intrinsic factor antibodies, Positive Schilling's test

Howell-Jolly bodies

Pernicious Anemia

TX of pernicious anemia

Give Vit B12 100-1000mcg IM QD 1wk, then Qwk 5-6wks, then Qmo; usually better in days and hematologically normal in ~2mos

Folic Acid deficiency

megaloblastic anemia, most frequently caused by inadequate intake (especially alcoholics)

Folic acid sources

green, leafy vegetables, liver, meats & some fruits, esp oranges and their juice

Etiologies of Folic acid deficiency

dietary deficiency, malabsorption, dialysis, folate antagonists, chronic hemolysis

Sx of Folic acid deficiency

Same as Vit B12 except NO neurological symptoms

Anemia of chronic disease

Mild normochromic/normocytic anemia initially, if persists become microcytic/hypochromic, most common type of anemia encountered among hospitalized pts

Etiologies of ACD

Chronic infection or inflammation, malignancies, chronic liver dz, ETOHism, chronic renal disease, endocrinopathies

ACD Labs

Normal MCV, MCHC, decreased Fe, Normal or increased ferritin, decreased or normal TIBC; new test hepcidin level increased

Hemolytic anemia

Congenital or acquired causes damage to RBC membrane leading to destruction of RBCs intravascularly or in macrophages of spleen or liver

Hemolytic anemia causes

immune system, chemicals, drugs, burns, venoms, thermal injury, RBC membrane defects, RBC enzymopathies, microorganisms and RBC destruction by passing thru fibrin threads

Autoimmune hemolytic anemia (AIHA)

due to cold agglutinins or warm-reactice Abs, Cold AIHA may be caused by infections, B cell malignancies; Warm AIHA may be caused by leukemias, lymphomas, myeloma, SLE and other autoimmune dzs, UC, viral infxns

Labs for Hemolytic anemia

Positive Coomb's test; increased retics, bilirubin, and urobilinogen; decreased haptoglobin, increased LDH; may see bizarre shapes and nRBCs on smear

Tx for hemolytic anemia

Treat underlying condition, steroids may be effective, RBC transfusion if markedly anemic, if cold AIHA avoid cold, if warm AIHA and no response to tx may need splenectomy

Microangiopathic hemolytic anemia

Mechanical chewing of RBCs, see abnormal RBC shapes on smear, caused by DIC, TTP, HUS, disseminated CA, PVD, scleroderma and collagen vascular dz and eclampsia or from mechanical heart valves and external trauma

RBC membrane defects

Hereditary spherocytosis, hereditary elliptocytosis, stomatocytosis, and xerocytosis

Hereditary spherocytosis

autosomal dominant D/O more common in northern Europeans caused by spectrin/ankyrin deficiency of RBC membrane causing fragility then spherocytes and hemolytic anemia

Glucose-6-Phosphate dehydrogenase Deficiency (G6PD)

X-linked recessive hereditary dz; G6PD important enzyme in RBC metabolism. More common in African, middle eastern, and south asians. Develop non-immune Hemolytic anemia to triggers.

Linked to favism

G6PD

Sickle Cell Anemia

Autosomal recessive d/o where Hgb S leads to hemolytic anemia and other problems; 8% of African-americans carry the gene

Sickle Cell Patho

when deoxygenated Hgb S forms polymers that damage RBC membrane. Hgb F retards process, Hypoxemia and acidosis worsen sickling

Sickle Cell onset

Onset in 1st year of life when Hgb F falls and Hgb S rises; hypoxemia and acidosis worsen sickling

Sickle Cell symptoms

hemolysis, jaundice, splenomegaly, gallstones and slow healing ulcers on leg; PAIN, Death is from organ failure and infection

Pain in Sickle Cell disease

Sickle cells occlude vessels causing infarction; affects bones, abdomen, chest and brain; priapism may occur

Autosplenectomy in sickle cell

Abnormal RBCs pool in spleen and undergoes repeated infarction that enlarges and function impaired then becomes fibrotic and shrinks, nonfunctioning spleen cannot filter/catch encapsulated organisms

Essentials to Diagnose Sickle Cell

Sickled RBCs on smear, +FH and lifelong hemolytic anemia, recurrent painful episodes, finding Hgb S on electrophoresis

Sickle Cell anemia Labs

Decreased MCV and MCHC, increased reticulocytes, Hemoglobin electrophoresis establishes dx, Howell-Jolly bodies and target cells, Nucleated RBCs

Tx of sickle cell dz

Primarily supportive (pain control, IV fluids, pnumococcal vaccine in childhood), treat complications, blood transfusion in crisis, Hydroxyurea increases Hgb F and decreases vaso-occlusive crises and painful episodes

Sickle Cell Trait

Clinically normal, Have 1 gene for formation of Hgb S & 1 gene for formation of normal Hgb; only symptomatic under extreme conditions (high altitudes, dehydration, extreme heat), Counseling for reproduction is needed, May protect against malaria

Thalassemias

Inherited group of anemias caused by absent or defective synthesis of one or more of the globin chains leading to defective Hgb production and damage to RBCs or their precursors by build-up of globin chain produced in excess

Thalassemia names

Named according to chain with reduced or absent synthesis (either alpha or beta of Hgb A)

Beta thalassemia major

AKA Cooley's anemia, most common in Mediterranean descent, no Beta chains produced. Physical growth and development delayed. Severe, transfusion dependent

Beta thalassemia major labs

decreased MCV, MCH and H/H but normal or increased RBC count, severe poikilocytosis and nRBCs on smear

Beta Thalassemia major in childhood

Fatal in late childhood and early adolescence as iron overload results from hypertransfusion and organ toxicity

Tx of Beta Thalassemia major

Need iron chelation (deferoxamine); may extend life to 4th decade, possible bone marrow tx

Beta thalassemia trait

decreased but not absent Beta chains; mild, asymptomatic hemolytic anemia; still have significant microcytosis/hypochromia with some poikilocytosis normal Fe studies

Alpha thalassemia

decreased or absent production of alpha chains, alpha syndromes depend on # of a alpha globin genes that are deleted; more common in chinese and southeast asians

Aplastic anemia

deficiency of hematopietic cells resulting in severe hypoplasia of bone marrow and pancytopenia; cause idiopathic but may be due to hereditary or exposure to drugs, infxns, chemicals, radiation

Aplastic anemia Labs

decreased WBCs, platelets, and RBCs; RBCs usually normocytic/normochromic but may be macrocytic; bone marrow aspirate shows hypocellularity

Anemia of pregnancy

From end of 1st trimester til ~20th week, there is progressive decrease in RBCs and H/H. Dilutional due to increase plasma volume versus a smaller increase in RBC mass. No tx needed

Sideroblastic anemia

Decreased Hgb synthesis secondary failure to incorporate heme into protoporphyrin to from Hgb; iron accumulates in RBC precursors in BM

Causes of Sideroblastic anemia

Often caused by chronic ETOHism, drug toxicity, and lead poisoning

Lead poisoning finding in RBCs

basophilic stippling

Labs of sideroblastic anemia

basophilic stippling of RBCs, increased serum iron, ferritin, and transferrin saturation, decreased TIBC; BM aspirate shows increased erythroid hyperplasia with ringed sideroblasts on Prussian blue stain

Packed RBCs

pRBCs used to correct for significant anemia, some pts require leukocyte-reduced pRBCs as WBCs are most common cause of febrile transfusion rxns

Most common transfusion rxn

Febrile nonhemolytic transfusion rxn due to small amount of WBCs in each unit of pRBCs

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