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Pathology Chapter 14 Review questions


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9 year old boy, generalized seborrheic skin eruption and fever. Treated previously for otitis media. Mild lymphadenopathy, hepatomegaly, splenomegaly. EM shows rodlike tubular Birbeck granules. What is the diagnosis
Langerhans cell histiocytosis. Skin eruptions, organomegaly, and lesion in the mastoid suggest infiltrates in multiple organs. Most often seen in children. Exophthalmos occurs in 50%. Hypothalamus and pituitary stalk involvement leads to diabetes insipidus (Hand-Schuller-Christian disease)
67 year old male with increasing weakness, fatigue, weight loss over five months. Decreasing vision, headaches and dizziness. Hands sensitive to cold. Generalized lymphadenopathy and hepatomegaly. Hyperproteinemia, protein 15.5, albumin 3.2. Marrow infil
Monoclonal IgM spike. Syptoms consistent with hyperviscosity syndrome, including visual disturbances, dizziness, and headaches. Has Raynaud phenomenon. Russell bodies are stored immunoglobulins in the cytoplasm. The patient has lymphoplasmacytic lymphoma (Waldenstrom macroglobulinemia). Neoplastic B cells differentiate to IgM producing cells. IgM molecules can agglutinate at low temperatures. Typically no leukemic phase
37 year old woman with cough and fever of one week's duration. Temp 38.3. Diffuse crackles in all lung fields. Chest radiograph shows bilateral extensive infiltrates. WBC 56,000 with 63% seg neut, 15% bands, 6% metamyelocytes, 3% myelocytes, 1% blasts, 8
Leukemoid reaction. Marked leukocytosis and immature myeloid cells in the peripheral blood can represent an exaggerated response to infection (leukemoid reaction) or can be a manifestation of chronic myelogenous leukemia (CML). Leukocyte alkaline phosphatase score is high in the more differentiated cell population seen in reactive leukocytosis.
What is hairy cell leukemia accompanied by in peripheral blood
Leukocytes that mark with tartrate-resistant acid phosphatase
Will a low leukocyte alkaline phosphatase score indicate Leukemoid reaction or CML
CML. CML will also have the Philadelphia chromosome
12 year old boy with abdominal distension and pain for three days. 7cm mass in the region of the ileocecal valve. Intermediate sized lymphoid cells, with nuclei having coarse chromatin, several nucleoli, many mitoses. Negative bone marrow biopsy. t(8:14)
Burkitt Lymphoma. Seen sporadically in young children, Africa, and HIV. Associated with translocations of MYC on chr8. Sporadic cases negative for EBV (Africa, HIV are positive). Typically extranodal
53 year old man with enlarged, nontender supraclavicular lymph node and enlargement of the Waldeyer ring of oropharyngeal lymphoid tissue. No hepatosplenomegaly. CBC normal, mild anemia. Lymph node shows replacement by a monomorphous population of large
Diffuse large B-cell lymphoma (DLBCL). Occurs in older persons and frequently manifests as localized disease with extranodal involvement, particularly the Waldeyer ring. CD19+10+ indicates B cells.
How does small lymphocytic lymphoma present
Widespread lymphadenopathy, liver and spleen enlargement, and lymphocytosis.
50 year old man with headache and dizziness +3 months. Generalized and severe pruritis. Dark stools. Afebrile, BP 165/90. No hepatosplenomegaly or lymphadenopathy. Stool has occult blood. Hg 22.3, Hct 67.1, MCV 94, platelets 453,000, WBC 7800. Diagnosis?
Polycythemia vera, a myeloproliferative disorder characterized by an increase RBC mass, with hematocrit concentration exceeding 60%. Results from transformation of of a multipotent stem cell. Pruritis and peptic ulceration result from histamine release from basophils.
50 year old man underwent chemotherapy seven years ago. Now reports fatigue and recurrent pulmonary and urinary tract infections. No masses, lymphadenopathy, hepatosplenomegaly. Hg 8.7, hct 25.2, MCV 88, platelets 67,000, WBC 2300, 15% neutr, 5% bands, 2
Myelodysplasia related to therapy for the previous tumor. Myelodysplasia is characterized by cellular marrow in which there are maturation defects in multiple lineages. Supported by presence of ringed sideroblasts, megaloblasts, abnormal megakaryocytes, and myeloblast in the marrow. 5q is a marker of post-therapy myelodysplasia
63 year old woman experiences burning sensation in hands and feet. Two months ago had an episode of swelling with tenderness in the right leg, followed by dyspnea and right-sided chest pain. Sleen and liver are enlarged. Hg 13.3, Ht 40.1, MCV 91, platele
Essential thrombocythemia. As will all myeloproliferative diseases, essential thrombocythemia occurs in a myeloid stem cell. Throbbing, burning pain caused by platelet aggregates that occlude small arterioles. Swelling in the leg represents phlebothrombosis, followed by pulmonary embolism with infarction
9 year old boy in Uganda with increasing pain and swelling on right side of face for 8 months. large, nontender mas involving the mandible, which deforms the right side of his face. No lymphadenopathy or spleneomegaly, afebrile. Intermediate sized lympho
EBV. Has endemic African variety of Burkitt lymphoma, a B-cell lymphoma that typically appears in the maxilla or mandible of the jaw.
23 year old man undergoing chemotherapy of acute lymphoblastic leukemia develops fever and ab pain for one week. Now has severe cought. Temp 38.4, crackles over all lung fields. Hg 12.8, hemat 39, MCV 90, platelet 221,000, WBC 16,475, 51% neut, 5% bands,
Parasite. The eosinophilia suggests a parasitic infestation. Immunocompromised persons can have superinfection and dissemination with strongyloidiasis
61 year old man with history of back pain +5 months. Productive cough with yellow sputum. Febrile, diffuse rales. No lymphadenopathy or splenomegaly. Culture grew Streptococcus pneumoniae. Serum Creatinine 3.7, urea nitro 35. Skull radiograph shows typic
Numerous plasma cells. This patient has multiple myeloma, which produces mass lesions of plasma cells in bone that leads to lysis and pain. Bone lesions produced by expanding masses of plasma cells. Bence Jones proteinuria can damage the tubules and give rise to renal failure. Often have infections with encapsulated bacteria because of decreased production of IgG
26 year old man with noted lumps in his neck that have been enlarging for 6 months. Group of enlarged, nontender right cervical lymph nodes. Scattered Reed-Sternberg cells, as well as macrophages, lymphocytes, neutrophils, eosinophils, and a few plasma c
Interleukin-5, which acts as an eosinophilic chemotactic factor to form an eosinophilic cellular component of the mixed cellularity and nodular sclerosis types of Hodgkin lymphoma.
53 year old woman experienced nausea with vomiting and early satiety for 7 months. Afebrile, no lymphadenopathy or hepatosplenomegaly. Hg 12.9, hct 41.9, platelet 263,000, WBC 8430. Loss of the rugal folds of the stomach over the fundus. Helicobacter pyl
MALT (marginal zone) lymphoma. Arise in middle aged adults at sites of autoimmune or infectious stimulation. Most common sites are thyroid (Hashimoto thyroiditis), salivary glands (Sjogren syndrome), Stomach (H pylori). Can transform to DLBCL. Cells correspond to the marginal B cells found at the periphery of stimulated lymphoid follicles.
18 month child wil seborrheic skin eruptions. Recurrent upper respiratory and middle ear infections with Streptococcus pneumoniae. Hepatosplenomegaly and generalized lymphadenopathy. 2 cm lytic lesion involving the right temporal bone. No anemia, thrombo
Histiocystes with Birbeck granules. The child has Letterer-Siwe dieases, a form of Langerhans cell histiocytosis. Birbeck granules are a distictive feature found in the cytoplasm of Langerhans cells.
Lymphoblasts that mark as T cells are seen in anterior mediastinal (thymic) masses in children with what disease
Acute lymphoblastic leukemia/lymphoma
When are Reed-Sternberg cells seen
Hodgkin disease
When are Ringed sideroblasts seen
Myelodysplastic syndromes
When are Sezary cells seen
Peripheral T-cell lymphoma/leukemias, which often involve the skin
50 year old woman with 3 month history of fatigue and dizziness. Recent syncopal episodes. Afebrile, BP 115/75. Marked pallor but no hepatosplenomegaly or lymphadenopathy. Hg 6.6, hct 19.9, platelet 199,800, WBC 4780, reticulocyte 0.1%. MCV, MCHC, serum
Thymoma. Patient has a rare disorder called pure RBC aplasia, characterized by selective suppression of the erythroid lineage in the bone marrow. Sometime associated with thymic tumor.
46 year old man with increasingly ruddy complexion for 4 months. Increasing fatigue. Afebrile, spleen tip palpable. Hg 21.3 hct 63.9, platelet 376,000, WBC 9210. Erythropoietin level is low What condition will produce this finding
Polycythemia vera. One of the myeloproliferative disorders, a neoplastic disorder of myeloid stem cells, which tend to differentiate predominantly along the erythroid lineage, giving rise to polycythemia. Require only small amounts of erythropoietin for survival.
41 year old man with fevers with chills and rigors for two weeks. Temp 39.2. Hg 13.9, hct 40.5, MCV 93, platelet 210,000, WBC 13,750, 75% seg neutr, 10% bands, 10% lymph, 5% mono. Bone marrow shows hypercellularity with a marked increase in myeloid precu
Chronic infections and chronic inflammatory conditions, such as a lung abscess. Can lead to expansion of the myeloid precursor pool in the bone marrow. Manifests as neutrophilc leukocytosis.
What drug can increase the release of marrow storage pool cells and diminish extravasation of neutrophils into tissues
37 year old man infected with HIV, admitted for ab pain +3 days. Ab distention and absent bowel sounds. Mass lesion involving the ileum. Firm, white mass infiltrated through the wall of the ileum. CD19+ lymphoid cells with prominent nuclei and nucleoli.
EBV. This patient has an extranodal infiltrative mass, made up of B cells. This is DLBCL. Tumors contain the EBV genome; immunosuppresion allows unregulated proliferation and neoplastic transformation.
What microorganism will be found in the spindle cells of Kaposi sarcoma and in body cavity B-cell lymphomas in patients with AIDS
Human herpesvirus type 8 (Kaposi sarcoma herpesvirus)
What microbe causes adult T-cell leukemia/lymphoma.
Human T-cell leukemia/lymphoma virus type 1
70 year old man experiencing increasing fatigue for 6 months. Nontender axillary and cervical lymphadenopathy; no hepatosplenomegaly. Hg 9.5, Hct 28, MCV 90, platelet 120,000, WBC 42,000. Monotonous population of small, round, mature-looking lymphocytes.
Clonal rearrangement of immunoglobulin genes. The patient has chronic lymphocytic leukemia, a clonal B-cell neoplasia in which immunoglobulin genes are rearranged and T cell receptor genes are in germline configuration.
What is t(9;22) a feature of
Chronic myeloid leukemia
What is the t(8;14) translocation typical in
Burkitt lymphoma
The t(14;18) translocation is a feature of what
Follicular lymphomas, which are distinctive B-cell tumors that involve the nodes and produce a follicular pattern. The lymphoma cells can be present in blood, but they do not look like mature lymphocytes
69 year old woman with increasing back pain for 1 month. Tenderness but no kyphosis or scoliosis. Tartial collapse of T11 and severaly lytic lesions with a rounded soap-bubble appearance in vertebrae. What characteristic feature will the laboratory find
Bence-Jones protiens in the urine from her multiple myeloma. The bone lesions result from bone destruction mediated by RANKL, a cytokine produced by the myeloma cells that activates osteoclasts
33 year old woman with generalized fatigue and night sweats for three months. Nontender right cervical lymphadenopathy. Microscopic pattern of thick bands of fibrous connective tissue with intervening lymphocytes, plasma cells, eosinophils, macrophages,
Nodular sclerosis, stage 1. The bands of fibrosis are typical of the nodular sclerosis type, commonly seen in young females. One lymph node involvement means stage 1.
15 year old boy with 10 day fever. Scattered petechial hemorrhages on trunck and extremeties. No enlargement of liver, spleen, or lymph nodes. Hg 13.2, hct 38.9, MCV 93, platelet 175,000, WBC 1850, 1% seg neut, 98% lymph, 1% mono. No abnormal bone marrow
Overwhelming bacterial infection. The major finding is marked granulocytopenia
7 year old boy with pain in right side of groin for past week. Painful, swollen lymph nodes in the right inguinal region. Node has large, variably sized, germinal centers contatin tingible body macrophages and numerous mitotic figures. Numerous parafolli
Acute lymphadenitis. Painful and acute enlarged nodes suggest a reactive condition and not a neoplastic process. Common in children.
15 year old boy with cough and high fever +4 days. Diffuse rales. Hg 14.8, hc 44.4, platelet 496,000, WBC 15,600. RBCs show marked anisocytosis and Howell-Jolly bodies. Culture grows Haemophilus influenzae. Diagnosis?
Prior splenectomy. This reduces immunity to encapsulated bacterial organisms. Spleen removes RBCs and removes inclusions such as Howell-Jolly bodies. Normally one third of platelets are sequestered in the spleen, and granulocytes are marginated in splenic sinusoids.
What does DiGeorge syndrome lead to
Cell-mediated immunodeficiency and increased viral, fungal, and parasitic diseases
69 year old man with lumps in the right side of his neck. Firm, nontender posterior cervical lymph nodes. Skin is intact and not erythematous. What will be the best evidence of malignant lymphoma of this node?
All lymphoid neoplasms are derived from a singel transformed cell and are therefore monoclonal. Presence of lymphoid cells positive for kappa, but not lambda, light chains could be seen.
4 year old listless for 1 week. Complains of pain, demonstrates irritability when his arms or legs are touched. Several large ecchymoses on right thigh and left shoulder. Hg 10.2, hct 30.5, MCV 96, platelet 45,000, WBC 13,990. Blasts that lack peroxidase
Acute lymphoblastic leukemia. Findings are characteristic of a childhood acute lymphoblastic leukemia of the pre-B cell type. Rapid expansion of marrow can lead to bone pain and tenderness.
49 year old woman with increasing weakness and chest pain over six months. Motor strenght dimisished with repetitive movement. Lab studies normal. Anterior mediastinal mass is removed, but it is difficult because if infiltrates surrounding structures. Co
Malignant thymoma. Can present with myasthenia gravis
62 year old man with fever and weight loss over six months. Nontender lymphadenopathy, palpable spleen tip. Hg 10.1, Hct 30.3, platelet 140,000 WBC 24,500, 10% seg neut, 1% bands, 86% lymph, 3% mono. Lymph node shows nodular pattern of small lymphoid cel
Mantel cell lymphoma. Has t(11:14) translocation, which activates the cyclin D1 (BCL1) gene
45 year old mane with recurrent fevers and weight loss for 5 months. Has cervical lymphadenopathy that become tender after a 6pack of beer. Effacement of the modal architecture by a population of small lymphocytes, plasma cell, eosinophils, and macrophag
Reed Sternberg cell. The patient has Hodgkin lymphoma, mixed cellularity type, which affects older men. Pain with alcohol consumption is a paraneoplastic phenomenon peculiar to Hodgkin disease
23 year old woman develops skin rash when outside. Has malar skin rash. Positive ANA test, titer 1:1024 with rim pattern. Positive anti-double-stranded DNA test. Hg 12.1, hct 35.5, MCV 89, platelets 109,000, WBC 4500. What would be seen in a WBC differen
Monocytosis. The patient has SLE, which can be accompanied by monocytosis.
When is eosinophilia typically seen
Allergic reaction, parasitic infections, Chronic myelogenous leukemia
Abnormal plasma cells that tend to retain the ability to secrete immunogloulins is characteristic of what
Multiple myeloma
Cells are found that have the BCR-ABL fusion gene from the reciprocal translocation t(9;22)(q34;11). The presence of this gene results in increased tyrosine kinase activity. What condition is this indicative of
Chronic myelogenous leukemia. This is the Philadelphia chromosome
64 year old man has inguinal, axillary, and cervical lymphadenopathy. Nodes firm and nontender. Nodular aggregates of small, cleaved lymphoid cells and larger cells with open nuclear chromatin, several nucleoli, and moderate amounts of cytoplasm. Marrow
Follicular lymphoma. Neoplastic B cells mimic a populatino of follicular center cells and hece produce a nodular or follicular pattern. Nodal involvement is often generalized, but extranodal involvement is uncommon. t(14;18) causes overexpression of BCL2
Many promyelocytes containing prominent aqurophilic granules and short, red, cytoplasmic rodlike inclusions calld Auer rods is characteristic of what
Acute promyelocytic leukemia. Will have t(15;17), with elaboration of abnormal retinoic acid receptor. Release of the granules can trigger DIC
Lymph nodes draining tissues involved by epithelial cancers are characteristics of type of chronic nonspecific lymphadenitis
Sinus Histiocytosis (Reticular Hyperplasia)
41 year old man with several bouts of pneumonia over the past year. Vague ab pain and dragging sensation. Marked splenomegaly. Hg 8.2, hct 24.6, MCV 90, platelet 63,000, WBC 2400. Small leukocytes with reniform nuclei and pale blue cytoplasm with threadl
Hairy cell leukemia, uncommon neoplastic disorder of B cells. Cells infiltrate spleen and marrow, resulting in pancytopenia. Two characteristics: Hairy projections from neoplastic leukocytes in peripheral blood smear and coexpression of B cell (CD19Cd20) and monocyte (CD11c) markers
7 year old boy with severe headache for one week. Tenderness on palpation of long bones, hepatosplenomegaly, and generalized lymphadenopathy. Petechial hemorrhages are present on the skin. Hg 8.8, hct 26.5, platelet 34,700, WBC 14,800. Bone marrow almost
Acute lymphoblastic leukemia. These are early pre-B cells that are absent the IgM heavy chain (u chain)
51 year old man visits because skin of face, neck and trunk have become scaly red. Intense itching and mild weight loss. Generalized exfoliative erythroderma. Generalized nontender lymphadenopathy. WBC 7940, 57% seg neut, 3% bands, 26% lymphocytes, 5% mo
Involvement of skin and the presence of lymphocytes with complex cerebriform nuclei in the skin and blood are features of cutaneous T-cell lymphomas. Do not confuse this with mycosis fungoides or Sezary syndrome.
65 year old man with fatigue, weight loss, night sweats, ab discomfort. Marked spleenomegaly, no lymphadenopathy. Hg 10, Hct 30, MCV 89, platelet 94,000, WBC 14,750, 55% neutr, 9% bands, 20% lymph, 8% mono, 4% meta, 3% myel, 1% eosin, 2 retic. Blood smea
Myelofibrosis with myeloid metaplasia. Neoplastic megakaryocytes secrete fibrogenic factors leading to marrow fibrosis. Extramedullary hematopoieses then takes place in the spleen
60 year old man with vague ab discomfort with bloating and diarrhea +6 months. Midab firm mass. Stool positive for occult blood. Mass involves the distal ileum and adjacent mesentery. Composed of sheets of large lymphoid cels with large nuclei, prominent
Diffuse Large B-cell lymphoma (DLBCL). Aggressive, but can be cured aggressively. Often involves extranodal sites, show large anaplastic lymphoid cells that involve the tissues diffusely.
45 year old man with weight loss, weakness, anorexia, fatigability +7 months. Splenomegaly. Hg 12.9, hct 38.1, MCV 92, platelets 410,000, WBC 168,000. Ph1 chromosome present. Diagnosis
Chronic myelogenous leukemia. Evolution of the disease is mainly along B-cell lines
14 year old boy with chest pain +5 days. Generalized lymphadenopathy. Clear lungs, mass in anterior mediastinum. Lymphoid cells with lobulated nuclei have delicate, finely stippled, nuclear chromatin. Scant cytoplasm, many mitoses. Express TdT, CD1,CD3,
Acute lymphoblastic leukemia. Age and location are typical of lymphoblastic lymphoma involving the thymus
60 year old woman with headaches and dizziness for 5 weeks. Mild spleenomegaly. Hg 21.7, hct 65%, platelet 400,000, WBC 30,000, 85% PMN leuk, 10% lymph, 5% mono. Smear has large platelets and nucleated RBCS. Erythropoietin undetectable, normal ferritin.
Polycythemia vera. Can lead to onset of marrow fibrosis with extensive extramedullary hematopoiesis in the spleen. 20% develops into myelofibrosis with myeloid metaplasia.
Children with frequent infections lack CD1a, CD2, CD3, CD4, CD8. What is the karyotypic abnormality
22q11.2. These cells mark as cortical lymphocytes in the thymus of a child. An absence of such cells can be seen in the DiGeorge anomaly with 22q11.2. Such patients can also have hypoplasia and congenital heart disease
Posterior meiastinal mass in 38 year old woman. Scattered large multinucleated cells, with prominent nucleoli that mark with CD15, and lymphocytes and macrophages separated by dense collagenous bands. What type of cell will be seen microscopically
Lacunar cells. These together with CD15 Reed-Sternberg cells indicate Hodgkin lymphoma. Lacunar cells have multilobed nuclei containing many small nucleoli
30 year old man with ulcerated, reddish-violet lesion on right forearm and nontender right axillary and left inguinal lymphadenopathy. Nodular left pleural mass. Right retroperitoneal mass. Large anaplastic cells, some of which contain horseshoe shaped n
Anaplastic large cell lymphoma, a form of T cell neoplasm. Extranodal and 2p23 rearrangement resulting in production of anaplastic lymphoma kinase
48 year old man has normal everything, except a small spike of gamma-globulin, which is IgG kappa. Plasma cells constitute 4% of bone marrow. Diagnosis?
Monoclonal gammopathy of undetermined significance. Characterized by presence of an M protein spike in the absence of any associated disease of B cells. Spike must be small, and Bence Jones proteinuria must be absent. Can progress to multiple myeloma in 20% of patients over 10-15 years
It is most likely that Reed Sternberg cells are derived from what
B cells. All RS cells will have clonal immunoglobulin gene rearrangements
22 year female with fatigue +2 months. No hepatosplenomegaly or lymphadenopathy. Mucosal gingival hemorrhages are noted. CBC shows hg 9.5, hct 28.2, MCV 94, platelet 20,000, WBC 107,000. Marrow 100% cellular with few residual normal hematopoietic cells.
Acute myelogenous leukemia. The high WBC count and the presence of peroxidase-positive blasts (myeloblasts) filling the marrow are characteristic of acute myelogenous leukemia

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