Clin Med Hematology

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______ is the MOST COMMON nutritional deficiency worldwide.
Epidemiology: The prevalence of iron deficiency is greatest among toddlers 1-2 years of age 7% and females 12-49 years of age 9-16%.
The prevalence was two times higher among non-Hispanic black and Mexican American females 10%.
Essentials of Diagnosis: Serum Ferritin <12µg/L (not iron). CAUSED BY BLEEDING IN ADULTS UNTIL PROVEN OTHERWISE! Response to iron therapy. Chronic aspirin or NSAID use may cause it even without a documented structural lesion. Menstrual blood loss in women; women with menorrhagia will almost always become iron deficient.
Signs and Symptoms: fatigue, tachycardia, palpitations, tachypnea, spoon nails. Severe changes: skin and mucosal changes, smooth tongue, brittle nails, cheilosis, PICA (Crave Ice).
Diagnosis:TIBC, Peripheral Blood smear: Anisocytosis, Poikoilocytosis, Microsytosis, hyprchromia, retic count.
Treatment: Oral iron: Ferrous Sulfate 325 mg TID. Return of crit levels halfway normal within 3 weeks with full return within 2 mo.
-Iron therapy should be continued for 3-6 mo after restoration.

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Iron Deficiency Anemia

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TICS:
T-Thalassemias
I-Iron Deficiency
C-Chronic Inflammation/disease
S-Sideroblastic – lead, drug, or hereditary (rare)

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MICROCYTIC

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Essentials of Diagnosis: Microcytosis out of proportion to the degree of anemia. Hemoglobin about 10.5. Positive family history or lifelong personal history of microcytic anemia. Abnormal red blood cell morphology with microcytes, acanthocytes, and target cells. In β-thalessemia, elevated levels of hemoglobin A² or F. Assess through hemoglobin electrophoresis
Pathophys: Hereditary; inadequate hemoglogin production. Reduced globin chain (α or β) synthesis causes reduced hemoglobin synthesis and eventually produces a hypochromic microcytic anemia.

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Thalassemias

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_______seen primarily in persons from southeast Asia and China, and less commonly in blacks. Normally adults have four copies of the α-globin chain.
When 3 α-globin genes are present, the patient is a silent carrier and hematologically normal.
When two α-globin genes are present, the patient is said to have α-thalessemia trait. These patients have a normal life expectancy and performance status with a mild microcytic anemia.
When only one α-globin chain is present, the patient has hemoglobin H disease. This is a chronic hemolytic (lysis-break apart cells) anemia variable severity.
Lab findings: mild anemia with hematocrits between 28-40%. The MCV is strikingly low (60-75 fL) despite the modest anemia and the RBC is normal or decreased. The peripheral smear shows microcytes, hypochromia, occasional target cells. THE RETIC COUNT AND IRON STORES ARE NORMAL.

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α-thalessemia

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Primary affects persons of Mediterranean origin (Italian and Greek) and to a lesser extent Chinese, other Asians, and blacks.
Affected children are normal at birth, but after 6 months, hemoglobin synthesis switches from hemoglobin F to hemoglobin A, severe anemia develops requiring transfusion.
Caused be mutations rather than deletions.
Defects cause reduced synthesis of hemoglobin.
The reduced B chains lead to excess A chains and this causes unstable and damaged red blood cell membranes. This leads to hemolysis. Marrow becomes hyperplastic. May cause bone deformities, osteopenia, and pathologic fractures.
Lab Findings: Modest hypochromic, microcytic anemia with MCV 55-75 fL. RBC normal or increased. The peripheral smear is mildly abnormal with target cells.

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B- Thalessemia

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Vitamin B 12 Deficiency, Macrocytic Anemia, Macro-ovalocytes and hypersegmented neutrophils on peripheral blood smear, Serum vitamin B12 level less than 100pg/ml

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Macrocytic Anemias

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The MOST COMMON cause of vitamin B12 deficiency is associated with ___________.
The disease is hereditary and rare clinically evident before the age of 35. These patients may have atrophic gastritis (gastric lining doesn’t work) and other autoimmune diseases as well as polyglandular endocrine insufficiency.

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Pernicious Anemia

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The HALLMARK of symptomatic vitamin B12 deficiency is _________.
In advanced cases the anemia may be severe with hematocrits as low as 10-15%. The megablastic state leads to glossitis and vague GI disturbances such as diarrhea, anorexia. Leads to a complex neurological syndrome.
Lab Findings: MCV is strikingly elevated. Smear is abnormal. Characteristic macro-ovalocyte. Hypersegmented neutrophils. Abnormally low vit B12 serum level.
Treatment: Parental therapy of 100µg Vitamin B12. Given daily 1x week for one month, followed by monthly for LIFE. Immediate response of well-being.

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megaloblastic anemia

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FOLIC ACID DEFICIENCY. Macrocytic anemia. Macro-ovalocytes and hypersegmented neutrophils on smear. Normal serum B12 levels (big difference). Reduced Folate levels in serum. MUST check both, folate and B12.
Lab findings: Megaloblastic anemia identical to that resulting from Vitamin B12. However, vitamin B12 level is normal. A folate level of less than 150 ng/mL is diagnostic of folate deficiency.
Treatment: Folic acid 1 mg/d orally. Total corrections within 2 mo.

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Macrocytic Anemia

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X-linked recessive disorder seen commonly in American black men. Episodic hemolysis in response to oxidant drugs or infection. Minimally abnormal smear. Avoid medications such as antimalarials, aspirin, sulfa drugs, and avoid eating fava beans.
Treatment: none, just avoid oxidant drugs.

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Glucose-6-Phosphate Dehydrogenase Deficiency (G6PD)

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Irreversible sickled cells on peripheral blood smear. Positive family history and lifelong history of hemolytic anemia. Recurrent painful episodes. Hemoglobin S is major seen on electrophoresis.
S & S: Onset is first year of life when hemoglobin F levels fall and the change is from γ-globin to β-globin production. Chronic hemolytic anemia produces jaundice, pigment, gallstones, splenomegaly, and poorly healing ulcers over the lower tibia.
Life expectancy: 40-50 years
Lab Findings: Chronic hemolytic anemia. WBC elevated. Hallmarks of hyposplenism-Howell-Jolly bodies.
Treatment: none; maintain pts on folic acid supplement. Pain meds.

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Sickle Cell Anemia

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PANCYTOPENIA. Decrease in everything, all cell lines! Everything is lowered, RBC/WBC/Platelets. No abnormal cells seen. Hypocellular bone marrow. Bone marros failure that arises from injury or abnormal expression of the stem cell. Causes: radiation, chemo, toxins, drugs, SLE, AIDS.
Lab findings: Anemia may be severe and is always associated with decreased reticulocytes.
Treament: Immediately refer for bone marrow biopsy. Supportive care; transfusions

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Aplastic anemia

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Anemia caused by decreased bone marrow production
Systemic, goes to veins and not just sight of tumor.
Treat with epoetin alfa injections weekly or darbepoetin alpha every other week or monthly. Keeps marrow producting RBC in chronic renal pts and this.

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Chemotherapy induced Anemia

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o Often due to idiosyncratic reactions to drugs.
Often due to the idiosyncratic reactions to drugs.
________ in the presence of normal bone marrow may be due to immunologic peripheral destruction, sepsis, or hyperslenism.
Treatment: Drugs discontinued, treat infections. Many cases of ideopathic or autoimmune neutropenia respond to myeloid growth factors, such as granulocyte colony-stimulating factor (G-CSF), Neulasta.

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Isolated Neutropenia