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1

What is the presentation of glucose–6–phosphate dehydrogenase deficiency?,

Sudden, severe, intravascular hemolysis; jaundice, dark urine, weakness, and tachycardia.

2

How is glucose–6–phosphate dehydrogenase deficiency diagnosed?,

High LDH, bilirubin, and reticulocyte count with a normal MCV and hemoglobinuria. Heinz bodies are inclusions in red cells. Definitive test is the G6PD level.

3

What is the treatment for glucose–6–phosphate dehydrogenase deficiency?,

Hydration and transfusion. Avoid oxidant stress.

4

What is aplastic anemia?,

Failure of bone marrow, resulting in anemia, leukopenia, and thrombocytopenia (pancytopenia). The marrow is empty.

5

What are the causes of aplastic anemia?,

Radiation, benzene, NSAIDs, chloramphenicol, alcohol, chemotherapeutics. Tb, lymphoma. Hepatitis, HIV, CMV, EBV, Parvovirus B19. The most common etiology is idiopathic.

6

What is the presentation of aplastic anemia?,

Bleeding from thrombocytopenia. Fatigue from anemia and infections from neutropenia. Pancytopenia on CBC. Marrow biopsy confirms.

7

What is the treatment for aplastic anemia?,

Bone marrow transplantation or immunosuppressive agents.

8

What is acute leukemia?,

Derangement of pluripotent stem cell, resulting in the destruction of marrow. Leukemic blasts crowd out marrow cells, resulting in pancytopenia. Blood cells lose the ability to mature and function.

9

What are the causes of acute leukemia?,

Most cases are idiopathic; radiation exposure, benzene, melphalan and etoposide, retroviruses.

10

What is the presentation of acute leukemia?,

Fatigue from anemia. Bleeding from thrombocytopenia. Infection from the underproduction of white blood cells.

11

Which type of anemia is more common in children?,

Acute lymphocytic leukemia (ALL) is more common in children. Acute myelogenous leukemia (AML) is more common in adults. Enlargement of the liver, spleen, and lymph nodes and bone pain are common at presentation.

12

How is acute leukemia diagnosed?,

WBC can be low, normal, or elevated. Thrombocytopenia, anemia; leukemic blasts in peripheral blood. Marrow biopsy >20% blasts confirms.

13

What are the markers of acute myelogenous leukemia?,

AML is characterized by Auer rods, myeloperoxidase, and esterase.

14

What are the markers of acute lymphocytic leukemia?,

ALL is characterized by common ALL antigen and terminal deoxynucleotidyl transferase (TdT).

15

What is chronic myelogenous leukemia (CML)?,

Massive overproduction of myeloid cells. Philadelphia chromosome is characteristic, producing tyrosine kinase.

16

What is the presentation of chronic myelogenous leukemia?,

Markedly elevated white blood cell count fatigue, night sweats, and low–grade fever. Abdominal pain from massive enlargement of the spleen. Bone pain.

17

How is chronic myelogenous leukemia diagnosed?,

Elevated WBC consisting of neutrophils. Leukocyte alkaline phosphatase score is diminished. Basophilia characteristic of CML and all myeloproliferative disorders such as polycythemia vera. Platelets elevated.

18

What is the treatment for chronic myelogenous leukemia?,

Imatinib which is direct inhibitor of the tyrosine kinase produced by the Philadelphia chromosome. 90% hematologic response.

19

What is chronic lymphocytic leukemia?,

Massive overproduction of mature; lymphocytes from production of B lymphocytes.

20

What is the presentation of chronic lymphocytic leukemia?,

CLL can often present as an asymptomatic elevation of white cells. 90% being over the age of 50. Fatigue, lethargy, and enlargement of lymph nodes. Infiltration of spleen, liver, and bone marrow.

21

How is chronic lymphocytic leukemia diagnosed?,

Older patient with a marked elevation in the white cell count with a marked lymphocytic predominance. The marrow is infiltrated with the leukemic lymphocytes with CD19 antigen.

22

What is multiple myeloma?,

Plasma cell overproduction, replacing the bone marrow; large quantities of functionless immunoglobulins.

23

What is the presentation of multiple myeloma?,

Bone pain. Pain in back, ribs; fractures. Radiculopathy. Encapsulated organisms (Pneumococcus, Haemophilus). Renal failure, anemia. Hypercalcemia: polyuria, polydipsia, altered mental. Weakness, pallor.

24

How is multiple myeloma diagnosed?,

Normochromic, normocytic anemia. Protein electrophoresis monoclonal immunoglobulin spike. Skull punched out lesions. beta2 microglobulin. Hypercalcemia from bone destruction. Elevated BUN, creatinine from kidney damage. Biopsy: 10% plasma cells.

25

What is Bence–Jones protein?,

Urinary Bence–Jones protein is nonfunctional gamma globulin found in multiple myeloma. It will not react with urine dipstick test.

26

What is the treatment for multiple myeloma?,

Bone marrow transplantation. Older patients should receive melphalan and prednisone.

27

What is monoclonal gammopathy of uncertain significance?,

Overproduction of immunoglobulin by plasma cells without manifestations MGUS is present in 1% > 50 and in 3% of those > age 70. Monoclonal immunoglobulin spike on electrophoresis. Treatment is not necessary.

28

What is Hodgkin disease?,

A neoplastic transformation of lymphocytes in the lymph node with Reed–Sternberg cells; spread to contagious areas of lymph nodes. Bimodal age peaks in the 20s and 60s.

29

What is the presentation of Hodgkin disease?,

Enlarged, rubbery, nonerythematous, nontender lymph nodes. symptoms are night sweats, weight loss, fevers. Pruritus; cervical, supraclavicular, axillary lymphadenopathy most common. Spleen, skin, gastric, lung, CNS.

30

What is the treatment for Hodgkin disease?,

Localized disease is managed with radiation. All patients with symptoms and stage III or stage IV disease are managed with chemotherapy.