A 40-year-old woman has had fatigue since 3 months. O/E, Pustules on face, hepatosplenomegaly. Lab. studies show Hgb 10.2 g/dl, Hct 31.1%, MCV 90 fL, WBC count 67,000/µL, platelet count 50,000/µL. PBS is shown. Labs- prolonged PT, PTT, thrombocytpenia, presence of D-dimers. Which of the following is the most likely chromosomal abnormality?
B. This is AML-M3; which shows a translocation b/w chr 15 and 17. RAR-PML fusion gene is formed that blocks the maturation of myeloid series at the promyelocyte stage.
A. BCL-2 is involved in follicular lymphoma.
C. 9;22 is seen in CML.
D. BCR-ABL is seen in CML.
E. C-MYC amplification is a feature of Burkitt lymphoma.
A 35-year-old man has had fatigue, fever, and episodes of epistaxis for past 3 months. O/E his T is 37.5 C. Lab. studies show Hb 12.5 g/dL, Hct 37.6%, MCV 89 fL, platelet count 170,000/µL, & WBC count 52,000/µL. Peripheral blood smear shows large blasts with Auer rods. What is the most likely diagnosis?
E. Acute myelogenous leukemia
(E) Auer rods are formed of cytoplasmic granules of myeloid blasts of AML and are a typical finding with AML.
Few myeloid blasts are present with CML.
Atypical lymphocytes of infectious mono do not have Auer rods.
Plasma cells do not have Auer rods. They do, however, often have a perinuclear halo (this is the prominent Golgi apparatus).
In CLL, the lymphocytes are small and mature. Lymphoid cells do not have Auer rods.
A 6-yr-old boy presents with fatigue and fever. O/E- marked pallor. Labs- anemia, thrombocytopenia, and leukocytosis. PBS shows 90% blasts that stain positive on IHC with TdT. What is your diagnosis?
TdT stains positive with immature lymphocytes.
A 5-year-old boy has become increasingly lethargic for past 2 months. O/E T- 37.3 C. Echymoses is noted on the skin of his lower legs. Hb 9.2 g/dL, Hct 27.8%, MCV 91 fL, platelet count 101,000/µL, WBC count 12,128/µL. BM biopsy shows 100% cellularity with replacement by primitive cells with large nuclei, delicate chromatin; indistinct nucleoli with scanty cytoplasm. These cells mark for CD10 (CALLA) antigen. Which of the following is the most likely diagnosis?
C. Acute lymphoblastic leukemia
(C) He has ALL, which typically occurs in children. The CALLA variety is common and responds well to therapy.
CD10 is a marker for lymphocytes.
B. Incorrect. HD is rare in the pediatric age range. Histologically, HD shows a prominent reactive lymphoid proliferation with neoplastic R-S cells.
D. Incorrect. He does not have infectious mononucleosis.
E. Incorrect. AML shows blasts with Auer rods and cells are positive for CD13 and CD33.
A figure skater who won gold medals at the 1928, 1932, and 1936 Winter Olympic games became progressively fatigued her late 50's, leading to a workup that revealed a CBC with Hb 10.1 g/dL (low), Hct 30.5%, MCV 90 fL, platelet count 89,000/uL (low), and WBC count 31,300/uL (high). From the peripheral blood picture shown here, the most likely diagnosis is:
Sonja Henie died from complications of chronic lymphocytic leukemia (CLL), in which there are increased numbers of circulating small, round, mature lymphocytes with scant cytoplasm seen in the peripheral blood smear. The cells express the CD5 marker and the pan B cell markers CD19 and CD20. Most patients have a disease course of 4 to 6 years before death, and symptoms appear as the leukemic cells begin to fill the marrow. In some patients, the same small lymphocytes appear in tissues; the condition is then known as small lymphocytic lymphoma. The lymphocytes seen in
infectious mononucleosis are “atypical lymphocytes,” which have abundant, pale blue cytoplasm that seems to be indented by the surrounding RBCs. The RBCs in iron deficiency anemia are hypochromic and microcytic, but the WBCs are not affected. Leukemoid reactions are typically of the myeloid type, and the peripheral blood contains immature myeloid cells. The WBC count can be very high, but the platelet count is normal.
Acute lymphoblastic leukemia is a disease of children and young adults, characterized by proliferation of lymphoblasts. These cells are much larger than the
cells in CLL and have nucleoli.
For past 4 months, a 62-year-old man has noted increasing fatigue and shortness of breath with minimal exercise. Physical examination shows non-tender cervical lymphadenopathy. Liver span measures 10 cm in right mid-clavicular line. Spleen is palpated 3 cm below left costal margin on inspiration. CBC shows WBC count 23,100/µL with 16 segmented neutrophils, 2 bands, 78 lymphocytes, and 4 monocytes, Hb 11.0 g/dL, Hct 36%, MCV 90, and platelet count 277,300/µL. Direct Coombs test is positive. Which of the following is the most likely diagnosis?
E. Correct. Most of the circulating cells are small, mature lymphocytes with CLL. Most persons with CLL are older adults.
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.
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.
C. Incorrect. With AML there are many immature myeloid forms, particularly myeloblasts.
D. Incorrect. Immature lymphoid forms-- lymphoblasts --are seen with ALL.
A 42-year-old man has had fevers for the past 4 weeks. On examination, he is febrile. Lab. studies show a Hb of 12.2 g/dL, Hct 37.1%, MCV 92 fL, platelet count 243,000/uL, and WBC count 75,000/uL (high). The WBC differential count shows 82 segs, 8 bands, 3 metamyelocytes, 1 myelocyte, 4 lymphocytes, and 1 monocyte. The leukocyte alkaline phosphatase (LAP) score is high at 130. Which of the following is the most likely diagnosis
B. Leukemoid reaction
(B) Correct. High WBC count with left shift (but no blasts) and high LAP score are consistent with leukemoid reaction. The term 'leukemoid' is used because there is an increased WBC count with immature forms similar to leukemia.
(A) Incorrect. With CML, the LAP score is low; the patient generally shows splenomegaly and the associated translocation is t(9;22) i.e. the 'Philadelphia chromosome'.
(C) Incorrect. Leuko-erythroblastic reaction shows immature erythroid as well as immature myeloid precursors. This is generally seen in patients with marrow infiltration or myelofibrosis with splenomegaly.
(D) Incorrect. AML-M5 shows monoblasts’ patients present with gingival infiltration of malignant cells.
(E) Incorrect. AML-M3 shows predominantly promyelocytes; multiple Auer rods and patients preent with DIC.
A 48-year-old man has experienced increasing malaise and difficulty concentrating at work for past 6 months. O/E he has splenomegaly but no lymphadenopathy. He is afebrile. Hb 12.0 g/dL, Hct 35.8%, MCV 92 fL, platelet count 390,000/uL, WBC count 190,000/uL with DLC 73 segs, 12 bands, 6 metamyelocytes, 2 myelocytes, 2 myeloblasts, and 5 lymphos. LAP score is decreased. Which of the following findings is most likely to be present in this patient?
C. Translocation between chromosomes 9 and 22
(C) He has CML in which there is a myeloproliferative process. Unlike acute myelogenous leukemia, blasts are uncommon in CML.
Multiple myeloma is not typically associated with an increased WBC count.
A.) Incorrect. This is not a sarcoma.
B.) Incorrect. This is not a metastatic adenocarcinoma.
(D) Incorrect. These features do not suggest a pancreatic or colon cancer.
(E) Incorrect. This is not a hepatocellular carcinoma or a yolk sac tumor.
Based upon the karyotype seen here, which of the following peripheral blood smear findings did this 43-year-old man most likely have:
(SORRY, THAT'S AS CLEAR AS I COILD GET THIS PICTURE)
This is a translocation, a t(9:22), the so-called 'Philadelphia chromosome' that is characteristic for chronic myelogenous leukemia (CML). There are few peripheral blasts, but basophilia and eosinophilia are common, and the leukocyte alkaline phosphatase score is markedly decreased.
A 60-yr-old female presents with headaches and dizziness. She has been taking omeprazole for heartburn. O/E- Plethoric cyanotic face. Hb- 21 g%, WBC- 30,000/cu. mm, platelets – 450,000/cu. mm. Serum erythropoietin is undetectable. Which of the following is the most likely diagnosis?
A. Polycythemia vera
The patient's symptoms of headaches and dizziness, along with the physical examination findings of a plethoric cyanotic face, suggest a high red blood cell count. The laboratory values of a hemoglobin level of 21 g%, a high white blood cell count of 30,000/cu. mm, and a normal platelet count are consistent with polycythemia vera. Additionally, the undetectable serum erythropoietin level supports the diagnosis of polycythemia vera, as this condition is characterized by the overproduction of red blood cells independent of erythropoietin.
A 62-yr-old male presents with vague abdominal pain and fatigue. O/E- Massive splenomegaly. Peripheral smear showed tear-drop cells and a leuko-erythroblastic reaction. The patient dies due to bacterial sepsis. BM biopsy at autopsy is shown. Which of the following is the most likely diagnosis?
A. Idiopathic myelofibrosis
The patient's presentation of vague abdominal pain, fatigue, massive splenomegaly, tear-drop cells, and leuko-erythroblastic reaction is consistent with idiopathic myelofibrosis. Idiopathic myelofibrosis is a myeloproliferative neoplasm characterized by fibrosis of the bone marrow, leading to extramedullary hematopoiesis in the spleen and liver. This can result in splenomegaly and the presence of immature blood cells in the peripheral blood, such as tear-drop cells and leuko-erythroblastic reaction. The patient's death due to bacterial sepsis is a common complication in idiopathic myelofibrosis, as the disease can cause immune dysfunction.
Which of the following best describes the pathogenesis of a patient with myelodysplastic syndrome?
B. Clonal stem cell defect
B. MDS is a clonal stem cell defect due to chemotherapy, radiation, benzene, smoking or viruses.
A. C-MYC is amplified in burkitt lymphoma
C. Globin chian synthesis is impaired in thalassemia
D. Functional asplenia is a feature of autosplenectomy that occurs in sickle cell anemia patients
A 37-year-old man infected with HIV for past 10 years has abdominal pain for past 3 days. O/E- Abdominal distension with diffuse tenderness and absent bowel sounds. Abdominal CT scan - mass lesion involving small intestine. He is taken to surgery, and an area of bowel obstruction in the ileum is removed. A near-encircling firm white mass 10 cm long and 3 cm in greatest depth that infiltrates thru’ bowel wall. Which of the following neoplasms is this man most likely to have?
C. Non Hodgkin B cell lymphoma
(C) Correct. Such lymphomas are typical for, as well as diagnostic of, AIDS in the setting of HIV infection. Extranodal involvement is common
(A) Incorrect. Multiple myelomas are usually found in bone.
(B) HD does not typically form a mass in the GIT.
(D) Metastatic disease is not common at his age, and adenocarcinomas are not increased in frequency with AIDS.
(E) Myelo-proliferative disorders are not a feature of AIDS and are unlikely to produce a mass lesion in bowel.
A 33-year-old woman has experienced low grade fevers, night sweats, and generalized malaise for the past 2 months. On examination, non-tender cervical and supra-clavicular LN is noted. A cervical LN biopsy shows occ. Reed-Sternberg cells along with large and small lymphocytes and bands of fibrosis. Which of the following is the most likely diagnosis?
B. Hodgkin disease
B) Reed-Sternberg cells are multinucleated with large nucleoli. Variants of them called lacunar cells are also seen with some forms of Hodgkin disease
A monomorphous proliferation of intermediate-sized lymphoid cells is seen with Burkitt lymphoma, often with accompanying macrophages.
Cat scratch disease is caused by the Rickettsia-like organism Bartonella henselae and leads to the appearance of stellate abscesses in lymph nodes.
Mycosis fungoides (despite the name) is not a fungal disease, but a form of cutaneous T-cell lymphoma.
A proliferation of plasma cells leads to multiple myeloma, usually detected by monoclonal immunoglobulin production
A 50-yr-old man presents with fever and generalized lymphadenopathy. LN biopsy is shown below. IHC would show staining for which of the following protein markers?
Follicular lymphoma occurs due to a t(14;18) translocation. Bcl-2 functions as an apoptosis inhibitor.
A 50-yr-old man presents with fever and generalized lymphadenopathy. Lymph node biopsy shows a follicular lymphoma. Which of the following is correct regarding the pathogenesis of the patient’s condition?
B. Inhibition of apoptosis
B. Correct. Follicular lymphoma occurs due to a t(14;18) translocation. Bcl-2 is over-expressed which functions as an apoptosis inhibitor.
A. Incorrect. This is seen in CML.
C. Incorrect. This is seen in BMurkitt lymphoma (amplification of C-MYC).
D. Incorrect. This is seen in AML-M3.
E. Incorrect. This is seen in Burkitt lymphoma.
A 10-year-old girl is noted to have increasing facial distortion for the past 8 months from a lesion involving her jaw. Physical examination shows a right mandibular mass. Biopsy shows many mitotic figures and many macrophages containing nuclear and cytoplasmic debris lying amidst proliferating lymphoid cells. Which of the following is associated with the pathogenesis of the patient’s condition?
A. Epstein-Barr virus
(A) 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
B. Incorrect. Hepatitis C virus infection may lead to development of hepatocellular carcinomas.
C. Incorrect. Herpes simplex viruses are not associated with development of neoplasms.
D. HIV is associated with development of non-Hodgkin lymphoma, including Burkitt-like lymphoma, but not the endemic African variety seen in the mandible.
A 32-year-old man has had worsening headaches for past 2 months. On examination, he is afebrile; no lymphadenopathy or hepatosplenomegaly seen. A head CT scan - 3 cm mass lesion to right of midline next to lateral ventricle. A brain biopsy shows diffuse large B cell lymphoma. A BM biopsy shows slightly decreased cellularity of all cell lines. Which of the following laboratory test findings is this patient most likely to have?
D. HIV-1 RNA of 8000 copies/mL
D) He has a cerebral lymphoma typical for AIDS with HIV infection.
A. Incorrect. Auer rods are typical for acute myeloid leukemia
B. Incorrect. Multiple myeloma does not typically involve the brain. Myeloma is a disease of older adults and is composed of plasma cells.
C. Incorrect. Macroglobulinemia can be seen in some lymphoplasmacytic lymphomas in the marrow (and possibly spleen, liver, and lymph nodes) of older adults.
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 55-yr-old man presents with recurrent epigastric pain. Gastric biopsy show malignant lymphoid cells infiltrating the stomach glands. Giemsa stain shows H. pylori. Which of the following is the most likely diagnosis?
D. Marginal zone lymphoma
Marginal zone lymphoma is associated with MALTomas, which can be seen in patients infected with H pylori.