a = new Array(234);
var f = "<FONT FACE=ARIAL SIZE=2>"
a[0] = ""
a[1] = f + "(A) Incorrect. Granulomas are unlikely to produce lytic lesions, and they are unlikely to be seen in association with bacterial sepsis."
a[2] = f + "(B) Incorrect. Chronic lymphocytic leukemia or lymphoma involving the marrow does not produce lytic lesions. Splenomegaly or lymphadenopathy is likely to occur, though."
a[3] = f + "(C) Incorrect. Reed-Sternberg cells are indicative of Hodgkin lymphoma, which typically does not produce lytic lesions."
a[4] = f + "(D) CORRECT. The findings suggest multiple myeloma. He has a markedly increased level of serum globulins. The renal failure and the increased risk for encapsulated bacterial infections is typical."
a[5] = f + "(E) Incorrect. An acute leukemia does not produce lytic lesions and is unlikely at his age."
a[6] = f + "(A) Incorrect. A monomorphous proliferation of intermediate-sized lymphoid cells is seen with Burkitt lymphoma, often with accompanying macrophages."
a[7] = f + "(B) CORRECT. Reed-Sternberg cells are multinucleated with large nucleoli. Variants of them called lacunar cells are also seen with some forms of Hodgkin lymphoma."
a[8] = f + "(C) Incorrect. Cat scratch disease is caused by the Rickettsia-like organism <I>Bartonella henselae</I> and leads to the appearance of stellate abscesses in lymph nodes."
a[9] = f + "(D) Incorrect. Mycosis fungoides (despite the name) is not a fungal disease, but a form of cutaneous T-cell lymphoma."
a[10] = f + "(E) Incorrect. A proliferation of plasma cells leads to multiple myeloma, usually detected by monoclonal immunoglobulin production."
a[11] = f + "(A) Incorrect. Atypical lymphocytosis in a young person suggests infectious mononucleosis, which is an infection."
a[12] = f + "(B) CORRECT. Infectious mononucleosis with Epstein-Barr virus (EBV) infection is typically acquired with close personal contact."
a[13] = f + "(C) Incorrect. You don't get infectious mononucleosis from door knobs or toilet seats either."
a[14] = f + "(D) Incorrect. A health care worker who acquires infectious mononucleosis from 'close personal contact' on the job is probably subject to disciplinary action."
a[15] = f + "(E) Incorrect. Atypical lymphocytosis at this age points to infectious mononucleosis as the likely etiology."
a[16] = f + "(A) Incorrect. This translocation is the 'Philadelphia chromosome' of chronic myelogenous leukemia. With CML, the LAP score is low."
a[17] = f + "(B) Incorrect. An anti-double stranded DNA suggests systemic lupus erythematosus, which is associated with leukopenia, not an increased WBC count."
a[18] = f + "(C) Incorrect. Decreased serum B12 or folate leads to a megaloblastic anemia, which may lead to anemia or occasionally leukopenia."
a[19] = f + "(D) Incorrect. The ketonuria with proteinuria suggests diabetes mellitus, which increases the risk for bacterial infections, but by itself does not greatly affect the WBC count."
a[20] = f + "(E) CORRECT. The high WBC count with left shift (but no blasts) and the high LAP score are consistent with a leukemoid reaction. The term 'leukemoid' is used because there is a markedly increased WBC count, with immature forms of WBCs, similar to leukemia."
a[21] = f + "(A) Incorrect. Platelets can be stored in the spleen and sequestered in a very large spleen, which can lead to thrombocytopenia."
a[22] = f + "(B) CORRECT. Increased numbers of red blood cell inclusions such as nuclear fragments (Howell-Jolly bodies) and degenerated hemoglobin (Heinz bodies) appear following splenectomy."
a[23] = f + "(C) Incorrect. Lack of a spleen means irregular RBCs and spherocytes are not removed, so that there is an increased variation in RBC size manifested as a high RDW."
a[24] = f + "(D) Incorrect. Leukopenia is more likely to occur with splenomegaly from trapping and sequestration than with splenectomy."
a[25] = f + "(E) Incorrect. The presence of nucleated RBCs relates more to abnormalities of bone marrow, such as myelophthisic processes. The spleen can be a site for extramedullary hematopoiesis."
a[26] = f + "(A) Incorrect. A lymphocytic lymphoma is a malignant monoclonal proliferation of lymphoid cells."
a[27] = f + "(B) Incorrect. Hodgkin lymphoma is a malignant lymphoproliferative process. Though many reactive lymphoid cells are present, there are diagnostic Reed-Sternberg cells."
a[28] = f + "(C) CORRECT. This is a typical history for infectious mononucleosis. The peripheral blood usually demonstrates atypical lymphocytosis. Liver involvement may lead to mild icterus."
a[29] = f + "(D) Incorrect. Though acute HIV infection can mimic infectious mono, it is not nearly as frequent. However, perform an HIV test if history for risk factors warrants it."
a[30] = f + "(E) Incorrect. Brucellosis is a chronic infection which is far less common than infectious mono. Granulomatous inflammation is usually present."
a[31] = f + "(A) Incorrect. Though a pronounced left shift can be seen with a leukemoid reaction, blasts should not be seen."
a[32] = f + "(B) CORRECT. The high WBC count with the blasts and Auer rods are very characteristic for an acute myelogenous leukemia."
a[33] = f + "(C) Incorrect. CLL is a disease of older adults, and the leukemic cells resemble small mature lymphocytes."
a[34] = f + "(D) Incorrect. ALL is typically a disease of young children, and the lymphoblasts do not have Auer rods or cytoplasmic granules positive for myeloperoxidase."
a[35] = f + "(E) Incorrect. There is a left shift and nucleated RBCs with leukoerythroblastosis, but not blasts."
a[36] = f + "(A) Incorrect. Diverticular disease may lead to chronic blood loss with iron deficiency anemia, in which the TIBC should be increased and the RBCs should be microcytic."
a[37] = f + "(B) CORRECT. He has findings of anemia of chronic disease, with a high (or normal) % iron saturation, increased iron stores, and normal soluble serum transferrin receptor. In iron deficiency, iron stores are diminished, % saturation decreased, and soluble serum transferrin receptor increased. Hepatitis C viral infection is a chronic disease."
a[38] = f + "(C) Incorrect. SLE can lead to cytopenias and hemolytic anemia and is more common in younger persons. He has no findings such as skin rash, arthralgia, or myalgia. Of the autoimmune diseases, rheumatoid arthritis is most likely to be associated with anemia of chronic disease."
a[39] = f + "(D) Incorrect. Atrophic gastritis should produce vitamin B12 deficiency with a megaloblastic anemia."
a[40] = f + "(E) Incorrect. This is a rare congenital anemia starting in childhood, with underlying defects in DNA repair, with associated anomalies such as organ hypoplasias."
a[41] = f + "(A) Incorrect. The small lymphocytic lymphoma (SLL) component of CLL may involve the lymph nodes, but there is a diffuse pattern of infiltration by small lymphocytes, not follicles. His normal WBC count is not consistent with CLL."
a[42] = f + "(B) CORRECT. Lymphadenopathy with malignant lymphoma is typically nontender, as contrasted with the lymphadenopathy of infections."
a[43] = f + "(C) Incorrect. As with most infections, the lymphadenopathy with Epstein-Barr virus infection is usually tender to palpation. Also, the lymphoid hyperplasia of mono is benign and polyclonal."
a[44] = f + "(D) Incorrect. Although there are numerous lymphocytes, occasional Reed-Sternberg cells or variants can be found. The lymphoid population of Hodgkin lymphoma is reactive."
a[45] = f + "(E) Incorrect. Reactive hyperplasia is polyclonal, not monoclonal."
a[46] = f + "(A) CORRECT. Myeloproliferative disorders, and myelofibrosis in particular, are known to cause massive splenomegaly."
a[47] = f + "(B) Incorrect. The spleen with sickle cell anemia becomes very small ('autosplenectomy')."
a[48] = f + "(C) Incorrect. A spleen will rarely be larger than 1000 gm in a patient (such as a chronic alcoholic with micronodular cirrhosis of liver) who has portal hypertension."
a[49] = f + "(D) Incorrect. The spleen may be slightly enlarged and tender, but is not massively enlarged with Epstein-Barr virus infection."
a[50] = f + "(E) Incorrect. There may be increased iron stores and the spleen may be some-what enlarged with hemochromatosis, but massive enlargement does not occur."
a[51] = f + "(A) CORRECT. Metastatic tumor involving marrow or marrow fibrosis reduces normal hematopoiesis leads to a peripheral 'leukoerythroblastic' picture with immature RBC's and WBC's in the peripheral blood smear."
a[52] = f + "(B) Incorrect. Macrocytosis is a common feature of alcoholism, not release of nucleated RBC's."
a[53] = f + "(C) Incorrect. Malabsorption could lead to iron or B12 or folate deficiencies, but the decreased marrow production of RBC's is not accompanied by release of nucleated RBC's."
a[54] = f + "(D) Incorrect. A hemoglobinopathy (such as sickle cell disease) severe enough to lead to release of nucleated RBC's would probably preclude survival to age 72."
a[55] = f + "(E) Incorrect. Chronic blood loss leads to iron deficiency, and this decreases marrow RBC production. Nucleated RBC's are not released in that circumstance."
a[55.1] = f + "(F) Incorrect. SLE and other autoimmune diseases may result in an autoimmune hemolytic anemia, with a positive Coombs test."
a[56] = f + "(A) Incorrect. DIC may produce peripheral hyaline thrombi, but typically follows as a complication of a serious disease process, not de novo."
a[57] = f + "(B) Incorrect. ITP can lead to thrombocytopenia, but an antibody to platelets is involved, and hyaline thrombi are not featured."
a[58] = f + "(C) CORRECT. The hyaline thrombi are typical for TTP, as is the pentad of fever, mental changes, renal failure, thrombocytopenia, and microangiopathic hemolytic anemia. Platelet transfusion is contraindicated."
a[59] = f + "(D) Incorrect. The hypercoagulable state that is seen with Trousseau syndrome (a paraneoplastic process) results mainly in large venous thrombi."
a[60] = f + "(E) Incorrect. Autoimmune hemolytic anemias result in hemolysis, but not thrombocytopenia."
a[61] = f + "(A) Incorrect. With a leukemoid reaction there is a marked increase in the WBC count with many neutrophils and metamyelocytes and myelocytes, but blasts are absent or rare. An infectious disease usually underlies a leukemoid reaction. Unlike CML, the leukocyte alkaline phosphatase is high with a leukemoid reaction."
a[62] = f + "(B) Incorrect. With CML there is a marked increase in the WBC count with many immature myelocytic forms, but not many blasts. Unlike leukemoid reaction, the leukocyte alkaline phosphatase is low with CML."
a[63] = f + "(C) Incorrect. With AML there are many immature myeloid forms, particularly blasts."
a[64] = f + "(D) Incorrect. Immature lymphoid forms-- lymphoblasts --are seen with ALL."
a[65] = f + "(E) CORRECT. Most of the circulating cells are small, mature lymphocytes with CLL. Most persons with CLL are older adults."
a[65.1] = f + "(F) Incorrect. SLE may be associated with Coombs positive hemolytic anemia, but is usually accompanied by cytopenias, not lymphocytosis, and not marked organomegaly."
a[66] = f + "(A) Incorrect. This disease is episodic and usually not manifested until an oxidizing drug such as primaquine is ingested that depletes RBC glutathione activity."
a[67] = f + "(B) CORRECT. Reduced beta-globin chain synthesis from beta-thalassemia leads to RBC microcytosis, hypochromia, ineffective erythropoiesis, and excessive iron absorption."
a[68] = f + "(C) Incorrect. The spleen is typically very small by age 16 with sickle cell disease as a consequence of multiple vaso-occlusive crises with infarction."
a[69] = f + "(D) Incorrect. Hereditary spherocytosis is usually a mild disease, and there is not excessive iron absorbtion."
a[70] = f + "(E) Incorrect. There can be hemolysis and splenomegaly with malaria, but excessive iron absorption is not usually a finding. This degree of severity of malaria is uncommon."
a[71] = f + "(A) Incorrect. Hypersplenism may account for cytopenias due to sequestration of circulating cells in the big spleen, but this does not account for a sudden drop in the platelet count."
a[72] = f + "(B) Incorrect. Chronic ITP is most common in women under the age of 40. There is an antibody directed at platelets. Bleeding is the most likely manifestation, with petechial hemorrhages noted. It is not likely that this disease would have an abrupt onset."
a[73] = f + "(C) Incorrect. DIC results from severe underlying diseases such as sepsis and shock. There is consumption of coagulation factors. Her PT and PTT remained normal."
a[74] = f + "(D) CORRECT. In about 5% of patients receiving heparin, antibodies develop to a complex of platelet factor 4 with heparin, and in 5 to 14 days there is a marked drop in platelet count. The feared complication is thrombosis ('white clot' syndrome), which can be arterial or venous, and in this patient a thrombotic stroke is likely to have occurred."
a[75] = f + "(E) Incorrect. TTP often has a pentad of neurologic abnormalities, thrombocytopenia (both of which she has), microangiopathic hemolytic anemia, renal failure, and fever (the last three are not present in this case)."
a[76] = f + "(A) Incorrect. Multiple myeloma is not typically associated with an increased WBC count."
a[77] = f + "(B) Incorrect. Although there is a 'left shift' with immature myeloid forms, a leukoerythroblastic reaction should not be accompanied by a low LAP score."
a[78] = f + "(C) CORRECT. He has chronic myelogenous leukemia (CML) which is a form of myeloproliferative process in which one or more cell lines (myeloid, erythroid, megakaryocytic) becomes increased. Unlike acute myelogenous leukemia, blasts are uncommon in CML."
a[79] = f + "(D) Incorrect. These features do not suggest aplastic anemia."
a[80] = f + "(E) Incorrect. A myelophthisic process from marrow infiltration by granulomatous inflammation can lead to the presence of immature myeloid cells in peripheral blood, but the LAP score should not be low."
a[81] = f + "(A) Incorrect. This oxidant drug can lead to increased RBC spherocytosis with RBC destruction in the spleen because glutathione metabolism is impaired in persons with G6PD deficiency."
a[82] = f + "(B) CORRECT. The findings point to hereditary spherocytosis. Parvovirus B19 infects erythroid precursors and can lead to an aplastic crisis in persons with hemoglobinopathies."
a[83] = f + "(C) Incorrect. Decreased oxygen tension is the mechanism behind the shape change of RBC's with sickle cell anemia."
a[84] = f + "(D) Incorrect. Exposure to cold can lead to hemolyis in patients with cold agglutinins, so called cold autoimmune hemolytic anemia."
a[85] = f + "(E) Incorrect. Transfusion of normal RBC's will tend to improve patients with RBC defects. A hemolytic crisis from mismatched blood is an extreme rarity."
a[86] = f + "(A) Incorrect. Few myeloid blasts are present with CML."
a[87] = f + "(B) Incorrect. The atypical lymphocytes of infectious mono do not have Auer rods."
a[88] = f + "(C) Incorrect. Plasma cells do not have Auer rods. They do, however, often have a perinuclear halo (this is the prominent Golgi apparatus)."
a[89] = f + "(D) Incorrect. In CLL, the lymphocytes are small and mature. Lymphoid cells do not have Auer rods."
a[90] = f + "(E) CORRECT. Auer rods are formed of the cytoplasmic granules of the myeloid blasts of AML and are a typical finding with AML."
a[91] = f + "(A) Incorrect. EBV infection is associated with infectious mononucleosis, a benign process, as well as some hematologic malignancies, but it is not typically associated with marrow hypercellularity."
a[92] = f + "(B) CORRECT. There is an increase in numbers of more than one cell line in the marrow. Paradoxically, there is bleeding because the platelets are functionally abnormal, even though there are lots of them."
a[93] = f + "(C) Incorrect. Drug reactions are typically toxicities associated with a decrease of one or more cell lines."
a[94] = f + "(D) Incorrect. This syndrome is accompanied by thrombocytopenia along with immunodeficiency."
a[95] = f + "(E) Incorrect. Though the marrow may become somewhat hypercellular with megaloblastic anemia, the cell counts in peripheral blood are not markedly increased."
a[96] = f + "(A) Incorrect. Certain oxidant drugs such as quinacrine or primaquine potentiate this disease."
a[97] = f + "(B) Incorrect. Certain drugs (such as aldomet) can cause this disease."
a[98] = f + "(C) Incorrect. Folate antagonists such as methotrexate can cause this disease."
a[99] = f + "(D) Incorrect. Some drugs can cause an aplastic crisis."
a[100] = f + "(E) CORRECT. Microcytic anemia is usually the result of an iron deficient state not associated with drug usage."
a[101] = f + "(A) Incorrect. Beta-thalassemias vary in severity, but generally are chronic conditions without hemolysis."
a[102] = f + "(B) Incorrect. Sickle cell anemia is marked by sickling crises brought on by decreased oxygenation, not drugs."
a[103] = f + "(C) Incorrect. Alpha-thalassemias tend to be mild in Africans because only 1 or 2 genes on separate members of a pair of chromosomes is involved."
a[104] = f + "(D) Incorrect. Hereditary spherocytosis is most likely to be seen in northern Europeans, and there can be aplastic crises brought on by parvovirus infection, not drugs.."
a[105] = f + "(E) CORRECT. He has glucose-6-phosphate dehydrogenase (G6PD) deficiency, which can result in a hemolytic anemia on exposure to oxidizing agents such as certain drugs such as antimalarials. This is an X-linked disorder. The Heinz bodies within the RBCs are formed from denatured hemoglobin."
a[106] = f + "(A) Incorrect. In autoimmune hemolytic anemias, the spleen is often the point of removal of the antibody-coated RBCs, and this increased activity increases the size of the spleen. In hemoglobinopathies with hemolysis, the spleen is also more active and increased in size."
a[107] = f + "(B) Incorrect. The portal hypertension accompanying portal fibrosis or cirrhosis with alcoholism leads to splenomegaly. Alcohol is a marrow toxin that can lead to anemia."
a[108] = f + "(C) Incorrect. Myelofibrosis leads to massive splenomegaly and marked extramedullary hematopoiesis."
a[109] = f + "(D) CORRECT. Though with ITP there are circulating antibodies to platelets that lead to platelet destruction, the spleen itself is usually not enlarged."
a[110] = f + "(E) Incorrect. By teenage years, persons with Hgb SS have undergone 'autosplenectomy' from the multiple infarctions, leading to a very small spleen."
a[111] = f + "(A) CORRECT. The increased MCV points to a macrocytic anemia such as a megaloblastic anemia that can also have hypersegmented PMN's from reduced numbers of nuclear divisions."
a[112] = f + "(B) Incorrect. Although the MCV can be increased with a pronounced reticulocytosis, it is usually not that high."
a[113] = f + "(C) Incorrect. The WBC count is usually high when an acute leukemia with blasts is present."
a[114] = f + "(D) Incorrect. Microcytic RBC's have a low MCV."
a[115] = f + "(E) Incorrect. Schistocytes, or fragmented RBC's, have a wide size distribution (high RDW)."
a[116] = f + "(A) Incorrect. Fragmented RBC's, called schistocytes, are indicative of a microangiopathic hemolytic anemia such as DIC."
a[117] = f + "(B) Incorrect. Nucleated RBC's suggest hemolysis (which is not consistent with his bilirubin level) or a disease infiltrating and replacing bone marrow."
a[118] = f + "(C) CORRECT. The most probable cause of a hypochromic, microcytic anemia in an older male is iron deficiency anemia."
a[119] = f + "(D) Incorrect. Hereditary spherocytosis is uncommon. With a normal bilirubin, hemolysis is probably not present."
a[120] = f + "(E) Incorrect. RBC inclusions suggest absence of the spleen."
a[121] = f + "(A) Incorrect. Sickle cell anemia is seen in persons of African and eastern Arabian ancestry. In perinatal life and in infancy, there is sufficient hemoglobin F to prevent complications."
a[122] = f + "(B) CORRECT. The form of alpha-thalassemia seen in Southeast Asia has two abnormal alpha globin genes on the same chromosome, so it is possible to inherit two bad sets, leading to a complete lack of alpha globin chain formation, so that fetal hemoglobin cannot be made, and there is a severe anemia."
a[123] = f + "(C) Incorrect. Hemoglobin E disease is seen in Southeast Asia, but typically causes a mild hemolytic anemia of adults."
a[124] = f + "(D) Incorrect. G6PD disease is most frequent in Africa and Mediterranean regions and can produce a hemolytic anemia when the RBC's are stressed by oxidants."
a[125] = f + "(E) Incorrect. Hereditary elliptocytosis may cause a mild anemia."
a[126] = f + "(A) Incorrect. The ineffective erythropoiesis leads to increased iron absorption with increased iron stores, but there is anemia."
a[127] = f + "(B) Incorrect. The hemolysis leads to increased iron recycling, since the red cells are not lost from the body, so iron stores are not significantly increased."
a[128] = f + "(C) Incorrect. Storage iron accumulates and is not effectively utilized with anemia of chronic disease. However, iron stores to not increase dramatically. An anemia is present."
a[129] = f + "(D) Incorrect. There is an increased RBC mass with primary polycythemia, but iron stores are not significantly increased."
a[130] = f + "(E) Incorrect. Ineffective megaloblastic erythropoiesis leads to increased iron absorption, but not dramatically, and a macrocytic anemia is present."
a[130.1] = f + "(F) CORRECT. Hereditary hemochromatosis results from increased iron absorbtion with markedly increased iron stores. The iron accumulation in tissues results in manifestations such as hepatomegaly, skin pigmentation, diabetes mellitus, heart disease, arthritis, and hypogonadism."
a[131] = f + "(A) CORRECT. The findings point to a leukemoid reaction that can be seen with infections such as tuberculosis."
a[132] = f + "(B) Incorrect. The findings are less likely to be chronic myelogenous leukemia than a leukemoid reaction. The Hgb and platelet counts are normal, and there is no eosinophilia or basophilia."
a[133] = f + "(C) Incorrect. He does not have multiple myeloma. Multiple myeloma is not characterized by an increased peripheral blood WBC count."
a[134] = f + "(D) Incorrect. An elevated D-dimer is a feature of microangiopathic hemolytic anemia, which is most likely to be seen with an elevated WBC count with acute promyelocytic leukemia."
a[135] = f + "(E) Incorrect. He does not have acute lymphocytic leukemia."
a[136] = f + "(A) Incorrect. CD10 is a marker for lymphocytes."
a[137] = f + "(B) Incorrect. Hodgkin lymphoma is rare in the pediatric age range. Histologically, Hodgkin lymphoma shows a prominent reactive lymphoid proliferation with neoplastic Reed-Sternberg cells."
a[138] = f + "(C) CORRECT. He has ALL, which typically occurs in children. The CALLA variety is common and responds well to therapy."
a[139] = f + "(D) Incorrect. He does not have infectious mononucleosis."
a[140] = f + "(E) Incorrect. The hematopoietic cellular proliferation in this case is lymphoid, not myeloid."
a[140.1] = f + "(F) Incorrect. In CLL, the lymphocytes are small and mature, with no blasts present. CLL is most likely to occur in older adults."
a[141] = f + "(A) Incorrect. An iron deficiency anemia would not be accompanied by nucleated RBC's. The MCV indicates that the RBC's are not microcytic."
a[142] = f + "(B) Incorrect. The MCV is not increased."
a[143] = f + "(C) Incorrect. Anemia of chronic disease is not accompanied by nucleated RBC's."
a[144] = f + "(D) Incorrect. It would be unusual for a patient with sickle cell anemia to live this long."
a[145] = f + "(E) CORRECT. She may have an autoimmune hemolytic anemia, or a leukemia or lymphoma may be present. The marked anemia has led to high output congestive heart failure with pulmonary edema."
a[146] = f + "(A) Incorrect. The presence of the mass suggests a renal cell carcinoma as the cause for the polycythemia."
a[147] = f + "(B) Incorrect. Erythroleukemia is a rare disease and it does not produce a renal mass."
a[148] = f + "(C) Incorrect. The blood loss from bleeding diatheses does not produce a rebound in red cell mass."
a[149] = f + "(D) Incorrect. Dehydration can temporarily increase the hemoglobin concentration, usually not to this degree; in this case the serum chemistries show no concentration effect."
a[150] = f + "(E) CORRECT. He has a secondary form of polycythemia. Renal cell carcinomas are known to secrete erythropoietin and lead to this paraneoplastic effect with polycythemia."
a[151] = f + "(A) CORRECT. There is evidence from the peripheral cytopenias for marked hypocellularity of the bone marrow with aplastic anemia. The spleen is of normal size."
a[152] = f + "(B) Incorrect. The spleen in CML can reach massive proportions."
a[153] = f + "(C) Incorrect. The spleen is often palpably enlarged with infectious mononucleosis, there may be mild anemia, but not marked pancytopenia."
a[154] = f + "(D) Incorrect. Large cell lymphomas tend to be localized masses that do not fill up the bone marrow to cause pancytopenia."
a[155] = f + "(E) Incorrect. There is splenomegaly with HS. Removal of the spleen in adults with HS leads to a decrease in problems resulting from spherocytosis."
a[156] = f + "(A) Incorrect. Hodgkin lymphoma affects mainly lymph nodes, and also liver, spleen, and bone marrow. It rarely involves tissues outside of the mononuclear phagocyte system."
a[157] = f + "(B) CORRECT. If these neoplastic cells circulate, it is known as Sezary syndrome."
a[158] = f + "(C) Incorrect. Burkitt lymphoma is often widespread, but in the abdomen or other visceral organs. The skin is rarely involved."
a[159] = f + "(D) Incorrect. ALL primarily involves the peripheral blood and bone marrow. Leukemic infiltrates can occur in skin, but are not common."
a[160] = f + "(E) Incorrect. Hairy cell leukemia leads to splenomegaly and pancytopenia. It does not involve skin. Hairy cell leukemia is a B-lymphocytic proliferation."
a[161] = f + "(A) Incorrect. CLL is a prolferation of mature lymphocytes. Splenomegaly is not common or not marked."
a[162] = f + "(B) Incorrect. Human T-cell lymphocytotrophic virus type I can lead to adult T-cell leukemias. This disease is endemic to Japan, the Caribbean, and parts of Africa."
a[163] = f + "(C) CORRECT. The proliferation of B-lymphocytes involves peripheral blood, bone marrow, and spleen. Splenomegaly is often present, but hepatomegaly is not common, and lymphadenopathy is rare with hairy cell leukemia."
a[164] = f + "(D) Incorrect. This storage disease due to a deficiency of the enzyme glucocerebrosidase leads to accumulation of large pale macrophages in the spleen. There is no lymphocytosis."
a[165] = f + "(E) Incorrect. Myelodysplastic syndromes lead to increase in immature myeloid cells in tissues and peripheral blood."
a[166] = f + "(A) Incorrect. Hemoglobin S leads to erythrocyte sickling and to the appearance of nucleated RBCs in the peripheral blood."
a[167] = f + "(B) Incorrect. Increased osmotic fragility is a feature of hereditary spherocytosis."
a[168] = f + "(C) Incorrect. A positive Coombs test suggests immune-related hemolysis."
a[169] = f + "(D) Incorrect. Though this is a hypochromic, microcytic anemia, the basophilic stippling suggests a toxic injury, not iron deficiency."
a[170] = f + "(E) CORRECT. She has lead poisoning. Lead inhibits incorporation of iron into heme, leading to elevated zinc protoporphyrin and free erythrocyte protoporphyrin. The diminished heme synthesis leads to a hypochromic, microcytic anemia, and there may be mild hemolysis. Basophilic stippling can be an indicator of toxic injury to RBCs."
a[171] = f + "(A) Incorrect. Myeloproliferative disorders are typically seen in adults and can involve myeloid, erythroid, and megakaryocytic cells lines, but not cells resembling histiocytes."
a[172] = f + "(B) Incorrect. Malaria may lead to hepatoplenomegaly, but not histiocytosis."
a[173] = f + "(C) Incorrect. Hodgkin lymphoma is typically seen in adults. The Reed-Sternberg cells should be numerous with lymphocyte depletion type and mark with CD15."
a[174] = f + "(D) CORRECT. The Langerhans cell histiocytoses include Letterer-Siwe disease (as in this case, it is typically a disseminated disease of children), and localized eosinophilic granuloma (often involving bone)."
a[175] = f + "(E) Incorrect. HIV infection produces loss of CD4 lymphocytes. Lymphadenopathy is possible, and large cell lymphomas can occur. There can be organomegaly in response to infections. However, histiocytic proliferations are not part of HIV infection."
a[176] = f + "(A) CORRECT. She has a cold autoimmune hemolytic anemia. Though this may be idiopathic or due to an infection (Epstein-Barr virus; <I>Mycoplasma</I>) a malignant lymphoma must be considered. Since the RBC's are being sequestered in the spleen with extravascular hemolysis, there is splenomegaly. A warm autoimmune hemolytic anemia will often produce intravascular hemolysis with hemoglobinemia that will more often lead to renal failure. There will more often be jaundice with intravascular hemolysis. Intravascular hemolysis will decrease haptoglobin more."
a[177] = f + "(B) Incorrect. SLE is more likely to be associated with a warm immunohemolytic anemia. SLE is less common with advancing older age."
a[178] = f + "(C) Incorrect. Pernicious anemia should be associated with a higher MCV (this woman's MCV could be explained by reticulocytosis). Megaloblastic anemias are not associated with immune hemolysis."
a[179] = f + "(D) Incorrect. Scleroderma can be associated with Raynaud phenomenon, but it is unlikely to be associated with an immunohemolytic anemia."
a[180] = f + "(E) Incorrect. Thalassemias can have ineffective erythropoiesis, but not immune-mediated hemolysis."
a[181] = f + "(A) Incorrect. Deletion mutations involve a loss of a single base pair, with frameshift."
a[182] = f + "(B) Incorrect. Duplication mutations involve an extra gene with more protein production."
a[183] = f + "(C) Incorrect. Insertion mutations involve a gain of a single base pair, with frameshift."
a[184] = f + "(D) CORRECT. Missense mutations involve a change in a single amino acid The defect in sickle cell anemia is a single amino acid substitution (valine for glutamic acid), but the globin chain is still made. The gene may less commonly be present in some Mediterranean and eastern Arabian populations. There is enough fetal hemoglobin at birth to prevent sickling with sickle cell disease."
a[185] = f + "(E) Incorrect. Nonsense mutations involve a change in a stop codon."
a[185.1] = f + "(F) Incorrect. Splice site mutations involve abnormalities at the intron-exon boundary."
a[185.2] = f + "(G) Incorrect. Triple repeat mutations involve increased tandem repeats."
a[186] = f + "(A) Incorrect. An elevated TdT is typical for acute lymphoblastic T cell leukemia/lymphoma."
a[187] = f + "(B) Incorrect. Multiple myeloma does not typically involve the brain. Myeloma is a disease of older adults and is composed of plasma cells."
a[188] = f + "(C) Incorrect. Macroglobulinemia can be seen in some lymphoplasmacytic lymphomas in the marrow (and possibly spleen, liver, and lymph nodes) of older adults."
a[189] = f + "(D) CORRECT. He has a cerebral lymphoma typical for AIDS with HIV infection."
a[190] = f + "(E) Incorrect. Though hairy cell leukemia is seen in middle aged males, it is accompanied by marked splenomegaly, and there is no CNS involvement."
a[191] = f + "(A) Incorrect. Plasma cell dyscrasias are not typically associated with hemolytic anemia."
a[192] = f + "(B) CORRECT. These findings all point to intravascular hemolysis that can be seen with warm autoimmune hemolytic anemia that can complicate autoimmune diseases such as SLE."
a[193] = f + "(C) Incorrect. Chronic viral infections may lead to anemia of chronic disease, not to hemolysis."
a[194] = f + "(D) Incorrect. Abnormal RBC shapes typically lead to increased RBC destruction in the spleen (extravascular), not intravascular hemolysis."
a[195] = f + "(E) Incorrect. B12 deficiency leads to anemia, but not to hemolysis. In this case the slightly elevated MCV is from reticulocytosis."
a[196] = f + "(A) Incorrect. Plasmacytomas are usually found in bone."
a[197] = f + "(B) Incorrect. Hodgkin lymphoma does not typically form a mass in the GI tract. It may be seen with some increased frequency with AIDS."
a[198] = f + "(C) CORRECT. Such lymphomas are typical for, as well as diagnostic of, AIDS in the setting of HIV infection. Extranodal involvement is common."
a[199] = f + "(D) Incorrect. Metastatic disease is not common at his age, and adenocarcinomas are not increased in frequency with AIDS."
a[200] = f + "(E) Incorrect. Myeloproliferative disorders are not a feature of AIDS and are unlikely to produce a mass lesion in bowel."
a[200.1] = f + "(F) Incorrect. Primary adenocarcinomas involve the small intestine are quite rare. Metastatic adenocarcinomas may occur, but his age is against that. Adenocarcinomas are not seen with increased frequency from HIV infection."
a[201] = f + "(A) Incorrect. Tear drop shaped RBCs are most likely to appear with myelophthisic processes such as myelofibrosis."
a[202] = f + "(B) Incorrect. Elliptocytosis can be an obscure, incidental finding."
a[203] = f + "(C) Incorrect. Target cells can be seen with liver disease and some hemoglobinopathies such as hemoglobin C disease."
a[204] = f + "(D) Incorrect. Macro-ovalocytes are seen with megaloblastic anemias."
a[205] = f + "(E) CORRECT. Howell-Jolly bodies and Pappenheimer bodies are seen within RBC's in splenectomized patients. A functioning spleen would normally remove such inclusions."
a[206] = f + "(A) Incorrect. Adenovirus is a type of herpesvirus that is not associated with development of neoplasms."
a[207] = f + "(B) Incorrect. Cytomegalovirus is."
a[208] = f + "(C) CORRECT. Burkitt lymphoma is endemic in Africa, mainly seen in children and young adults, and EBV infection is implicated in the pathogenesis of this B-cell lymphoma. Although Burkitt lymphoma is one of the fastest growing neoplasms, half of cases do respond to chemotherapy with long-term survival."
a[209] = f + "(D) Incorrect. Hepatitis C virus infection may lead to development of hepatocellular carcinomas."
a[210] = f + "(E) Incorrect. Herpes simplex viruses are not associated with development of neoplasms."
a[210.1] = f + "(F) Incorrect. HIV is associated with development of non-Hodgkin lymphoma, including Burkitt-like lymphoma, but not the endemic African variety seen in the mandible."
a[210.2] = f + "(G) Incorrect. HTLV-1 is endemic to parts of the Caribbean and Japan. It is associated with development of adult T-cell leukemia following decades of infection."
a[211] = f + "(A) Incorrect. Hemophilia A and B are disorders of coagulation factors VIII and IX respectively, and are X-linked recessive. Though it is possible that the girl could have inherited an abnormal gene on each X chromosome, or have random X inactivation, this is unlikely. Hemophilia often leads to hemarthroses with joint problems. Platelets are not affected."
a[212] = f + "(B) Incorrect. ATIII deficiency is an uncommon cause for coagulopathy. ATIII interact with heparin-like molecules to inactivate thrombin. Thus, ATIII deficiency leads to thrombosis, not bleeding."
a[213] = f + "(C) CORRECT. Glanzmann thrombasthenia is an autosomal recessive disorder from abnormalities in platelet glycoprotein IIb or IIIa, which form a receptor that is required for platelet aggregation, for uptake of fibrinogen into platelets, and clot retraction. This is a qualitative platelet defect; there are normal numbers of circulating platelets."
a[214] = f + "(D) Incorrect. SLE can produce immunohemolytic anemia. Hemolysis leads to increased RBC production in marrow with more reticulocytes that are larger in size. SLE can be associated with thrombocytopenia, not qualitative platelet defects. SLE can be associated with antiphospholipid antibodies, which disrupt coagulation leading to either thrombosis or hemorrhage, but not to platelet dysfunction."
a[215] = f + "(E) Incorrect. Von Willebrand disease may have similar clinical features from abnormal bleeding from abnormally functioning plateletts, with decreased VWF, which stabilizes factor VIII. It is autosomal dominant. Platelet function studies will show an abnormal response to ristocetin."
a[216] = f + "(A) Incorrect. CLL can be associated with hemolytic anemia, which is not present here. CLL is usually seen in older persons."
a[217] = f + "(B) Incorrect. Peptic ulcer disease should lead to blood loss with iron deficiency and a low MCV."
a[218] = f + "(C) Incorrect. Lead poisoning interferes with heme synthesis, leading to a hypochromic microcytic anemia. There may be mild hemolysis. RBCs can show basophilic stippling, a feature of toxic marrow damage."
a[219] = f + "(D) CORRECT. There is mild macrocytosis with normal B12 and folate, without evidence for significant hemolysis. Liver disease can lead to RBC membrane abnormalities, with the presence of a few target cells on the peripheral blood smear. Chronic alcohol abusers may also have poor diets lacking in folate. An MCV >100 fL is much more likely to result from megaloblastic anemia (B12 or folate deficiency)."
a[220] = f + "(E) Incorrect. Spherocytosis leads to a lower MCV."
a[221] = f + "(A) Incorrect. The MCV is normal. In iron deficiency, the marrow could not put out nucleated RBC's."
a[222] = f + "(B) CORRECT. The immature WBC's and RBC's in the peripheral blood are typical for leukoerythroblastosis. He probably has widely metastatic prostate cancer to bone marrow."
a[223] = f + "(C) Incorrect. No blasts are present, and the WBC count is not elevated."
a[224] = f + "(D) Incorrect. DIC could decrease the platelet count, but it would not explain the immature WBC's and RBC's in the peripheral blood."
a[225] = f + "(E) Incorrect. The WBC count should be markedly increased in a leukemoid reaction."