Advanced Hematology Exam #2

An 80-year-old woman with Lupus Erythematosus has increasing complaints of being "tired all the time". Her blood work results are as follows: RBC: 3.30 X 10^6/cmm, Hgb: 8.2 g/dL, Hct: 26.0 %, Serum Iron; 45 mg/dL, TIBC: 150 µg/dL. What is the percent transferrin saturation? A. 10 % B. 30 % C. 50 % D. 75 %

30 %

The marrow serves as an iron trap. It receives ___________ % of the total cardiac output, but it extracts __________% of the plasma iron. A. 25%; 50% B. 50%; 50% C. 50%; 90% D. 5%; 85%

5%; 85%

Serum iron levels in Sideroblastic anemia are: A. 0 - 50 µg/dl B. 50 - 150 µg/dl (normal) C. > 150 µg/dl D. Cyclic in a 24 hour period

> 150 µg/dl

Match the porphyria with the enzyme deficient (C) in the pathway below. Porphobilinogen deaminase

Acute Intermittent Porphyria (AIP)

Acute intermittent porphyria is due to: A. An inherited defect in the amount of porphobilinogen (PBG) B. An inherited deficiency of the enzyme uroporphyrinogen synthetase C. An acquired inhibition of the enzymes Ala-dehydrase, ferrochelatase, and coproporphyrinogen D. A defect in iron metabolism

An inherited deficiency of the enzyme uroporphyrinogen synthetase

An 80-year-old woman with Lupus Erythematosus has increasing complaints of being "tired all the time". Her blood work results are as follows: RBC: 3.30 X 10^6/cmm, Hgb: 8.2 g/dL, Hct: 26.0 %, Serum Iron; 45 mg/dL, TIBC: 150 µg/dL. This patient's medical history, lab results, and transferrin saturation values are characteristic of: A. Anemia of hypothyroidism B. Iron deficiency anemia C. Sideroblastic anemia D. Anemia of chronic disorders

Anemia of chronic disorders

Which of the following anemias would present with RBC target cells? A. Anemia of liver disease B. Anemia of renal disease C. Aplastic anemia D. Anemia of chronic disorders

Anemia of liver disease

A characteristic and distinguishing RBC-morphological finding found in lead poisoning is __________________. A. Howell Jolly Bodies B. Polychromatophilia C. Basophilic Stippling D. Dacrocytes

Basophilic Stippling

Hemochromatosis can occur from all of the following causes, EXCEPT: A. Multiple transfusions B. Chronic blood loss C. Sideroblastic anemia D. Overdose of iron pills

Chronic blood loss

Match the porphyria with the enzyme deficient (D) in the pathway below. Uroporphyrinogen III Cosynthetase

Congenital Erythropoietic Porphyria

Match the porphyria with the enzyme deficient (E) in the pathway below. Uroporphyrinogen I Oxidase

Cutaneous Hepatic Porphyria

The earliest laboratory indication of developing IDA is which of the following results? A. Microcytic RBCs B. Decrease in plasma ferritin C. Decrease in plasma iron D. Decrease in marrow iron stores

Decrease in plasma ferritin

The molecular form of iron most efficiently absorbed from digested food across the mucosal cell barrier is: A. Ferric B. Fe2+ C. Ferritin D. Fe3+

Fe2+

A serum iron of 227 µg/dl and a transferrin saturation of 85% is related to which disease? A. Polycythemia vera B. Aplastic anemia C. Hemochromatosis D. Refractory anemia

Hemochromatosis

Match the porphyria with the enzyme deficient (A) in the pathway below. Coporphyrinogen-III Oxydase

Hereditary Coproporphyria

A 26-year-old female presented with a moderate anemia and persistent mouth ulcers and sore tongue. The peripheral blood smear represented by image below, is an example of her blood picture (Hypochromic bloodcells). Based on her symptoms and blood picture, this patient most likely has which of the following disorders? A. IDA B. Anemia of Chronic disorders C. Aplastic anemia D. Anemia of hypothyroidism

IDA

The differential diagnosis of microcytic/hypochromic anemias includes Iron Deficiency Anemia, Anemia of Chronic Disorders, Sideroblastic anemia, and Thalassemia. Of the four disorders, which one typically presents with an increased TIBC and serum ferritin values below normal? A. Iron Deficiency Anemia B. Anemia of Chronic Disorders C. Sideroblastic anemia D. Thalassemia

Iron Deficiency Anemia

A 26-year-old female presented with a moderate anemia and persistent mouth ulcers and sore tongue. The peripheral blood smear represented by image below, is an example of her blood picture (Hypochromic bloodcells). What would be the next lab test ordered to give a definitive diagnosis? A. Bone marrow B. Erythropoietin C. Iron and TIBC D. Prussian blue stain

Iron and TIBC

All of the following statements are TRUE concerning iron metabolism, EXCEPT: A. Iron is stored in all normoblasts and macrophages as hemopexin B. Dietary iron is absorbed in the duodenum in the ferrous (Fe 2+) form but transported in the circulation and stored in the ferric form (Fe3+) C. The majority of iron used in Heme synthesis is "recycled" iron from degraded Hgb from intravascular and extravascular hemolysis pathways D. 2/3 of the body iron is found in normoblasts and RBCs

Iron is stored in all normoblasts and macrophages as hemopexin

An 80-year-old woman with Lupus Erythematosus has increasing complaints of being "tired all the time". Her blood work results are as follows: RBC: 3.30 X 10^6/cmm, Hgb: 8.2 g/dL, Hct: 26.0 %, Serum Iron; 45 mg/dL, TIBC: 150 µg/dL. Morphologically, the anemia you identified on this particular patient, can be classified as: A. Normocytic/Normochromic B. Normocytic/Hypochromic C. Microcytic/Hypochromic D. Macrocytic/Normochromic

Microcytic/Hypochromic

Which one of the following lists of laboratory findings would be most characteristic of an anemia of chronic inflammation? A. Normocytic/hypochromic, high serum iron, increased TIBC, decreased ferritin levels, decreased Hgb. & Hct. B. Microcytic/hypochromic, normal serum iron, decreased TIBC, normal ferritin levels, decreased Hgb. & Hct. C. Normocytic/normochromic, low serum iron, decreased TIBC, increased ferritin levels, decreased Hgb. & Hct. D. Macrocytic/normochromic, low serum iron, normal TIBC, decreased ferritin levels, normal Hgb. & Hct.

Normocytic/normochromic, low serum iron, decreased TIBC, increased ferritin levels, decreased Hgb. & Hct.

A genetic abnormality in a gene on chromosome 6 associated with an increased iron absorption by the small intestinal mucosal cell is: A. Pyridoxine (B6) deficiency B. Iron deficiency anemia C. Primary Familial Hemochromatosis D. Diamond-Blackfan anemia

Primary Familial Hemochromatosis

All of the following agents have been known to cause Hypersensitive Aplastic Anemia, EXCEPT: A. Chloramphenicol B. Radiation C. DDT D. Some antihistamines

Radiation

In general, Porphyrias result in the following conditions, EXCEPT: A. Result from an inherited deficiency in ALA-synthetase B. Can often be clinically related to peripheral neuritis, psychosis, and photosensitive skin C. Are often inherited disorders of a certain enzyme in the heme biosynthetic pathway, resulting in an increase in ALA-synthetase and the porphyrin intermediates prior to the enzyme block D. Result in a microcytic/hypochromic anemia

Result from an inherited deficiency in ALA-synthetase

Match the porphyria with the enzyme deficient (B) in the pathway below. Ferrochetalase

Variegate Porphyria