Parcialito 12 - Hematolinfoide Flashcards
1
Q
Average volume per cell, expressed in femtoliters.
A
Mean cell volume (MCV)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
2
Q
A reduction in the oxygen-transporting capacity of blood.
A
Anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 422
3
Q
The average content of hemoglobin per red cell, expressed in picograms.
A
Mean cell hemoglobin (MCH)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
4
Q
The average concentration of hemoglobin in a given volume of packed red cells, expressed in g/dL.
A
Mean cell hemoglobin concentration (MCHC)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
5
Q
The coefficient of variation of red cell volume.
A
Red cell distribution width (RDW)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
6
Q
Anemia of acute blood loss is described as ______.
A
Normocytic, normochromic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 423
7
Q
Life span of a normal red cell.
A
120 days(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
8
Q
Anemia characterized by an increased rate of cell destruction, a compensatory increase in erythropoeisis that results in reticulocytosis, and retention of products of cell destruction, including iron.
A
Hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
9
Q
A circulating protein that binds and clears free hemoglobin.
A
Haptoglobin(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
10
Q
Hemolysis that can result from mechanical trauma, or biochemical or physical agents that damage the red cell membrane.
A
Intravascular hemolysis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
11
Q
Hemolysis which takes place largely within phagocytic cells of the spleen and liver.
A
Extravascular hemolysis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
12
Q
This disorder is characterized by an intrinsic defect in the red cell membrane, that renders the cells spheroidal, less defomable and vulnerable to splenic sequestration and destruction.
A
Hereditary spherocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 424
13
Q
Small, dark nuclear remnants seen within red cells in PBS of hereditary spherocytosis.
A
Howell-Jolly bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425
14
Q
On PBS, red cells are spherical which lack central pallor, and they show increased osmotic fragility when placed in hypotonic salt solutions.
A
Hereditary spherocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425
15
Q
Structural proteins that are defective in hereditary spherocytosis.
A
Spectrin and ankyrin(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 425
16
Q
This results from substitution of valine for glutamic acid at the 6th position of the B-globin chain, producing HbS.
A
Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 426
17
Q
Bizarre, elongated, spindled or boat-shaped cells on PBS.
A
Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 427
18
Q
Prominent cheekbones and changes in skull resembling a “crew-cut” skull x-ray.
A
Sickle cell anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 427
19
Q
Patients with sickle cell disease are predisposed to infections caused by these type of bacteria.
A
Encapsulated bacteria(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
20
Q
Treatment for sickle cell disease by increasing levels of HbF.
A
Hydroxyurea(TOPNOTCH)
21
Q
Treatment for sickle cell disease by increasing levels of HbF.
A
Hydroxyurea(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
22
Q
Feared complication of sickle cell disease which can be trigerred by pulmonary infections or fat emboli from necrotic marrow that secondarily involve the lung.
A
Acute chest syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
23
Q
Major complication of sickle cell disease which occurs in the setting of acute chest syndrome, causing ischemic injury to the CNS.
A
CNS stroke(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
24
Q
Represents a sudden but usually temporary cessation of erythropoeisis, usually trigerred by parvovirus B19 infections in patients with sickle cell disease.
A
Aplastic crises(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 428
25
In beta thalassemia, an individual who inherits one abnormal allele (out of 2) has this asymptomatic to mildly symptomatic condition.
B- Thalassemia minor/trait(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429
26
Individuals with B-thalassemia who inherit two abnormal alleles, with severe anemia requiring regular blood tranfusions.
B- Thalassemia major(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429
27
Red cells with a central, dark-red puddle due to collection of hemoglobin.
Target cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
28
Target cells are often seen in this condition.
B-thalassemia minor(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
29
In the PBS of this condition, nucleated red cells (normoblasts) are seen, which reflect underlying erythropoeisis.
B-thalassemia major(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
30
Anemia of beta thalassemia.
Microcytic, hypochromic(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 430
31
Disease caused by deletion of 3 alpha globin genes.
Hemoglobin H disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431
32
Condition caused by deletion of 1 alpha globin gene.
Silent carrier(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431
33
Condition caused by deletion of 2 alpha globin genes.
Alpha thalassemia trait(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 431
34
Condition caused by deletion of all four alpha globin genes.
Hydrops fetalis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 429
35
Precipitates of denatured globin seen in RBC's.
Heinz bodies(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
36
Heinz bodies are seen in the blood smear of this condition.
G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
37
Bite cells are seen in ________.
G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
38
A rare disorder of unknown etiology, causes hemolytic anemia which results from an acquired membrane defect secondary to a mutation that affects myeloid stem cells. Hemolysis occurs during sleep.
Paroxysmal nocturnal hemoglobinuria(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
39
Hemolysis caused by IgG or IgA antiodies that are active at 37 degC, which results in opsonization of red cells by the autoantibodies.
Warm antibody immunohemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
40
Anemia caused by low-affinity IgM which bind to red cell membranes only at temp
Cold antibody immunohemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
41
Anemia observed in a variety of pathologic states, in which small vessels become particularly obstructed.(e.g. DIC, malignant HTN, SLE, etc.)
Microangiopathic hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
42
Schistiocytes, burr cells, helmet cells, triangle cells are seen in this condition.
Microangiopathic hemolytic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 433
43
X-linked disorder in which red cells are unusually susceptible to damage cause by oxidants.
G6PD Deficiency(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 432
44
Red blood cells in iron deficiency anemia.
Microcytic, hypochromic(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 436
45
Diagnostic criteria for iron deficiency anemia:____ Ferritin____Serum iron level____Transferrin saturation____Total Iron Binding Capacity (TIBC)
Low ferritin
Low serum iron levels
Low transferrin saturation
Increased TIBC
(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 435
46
Diagnostic criteria for anemia of chronic disease:____ Ferritin____Serum iron level____Transferrin saturation____Total Iron Binding Capacity (TIBC)
Increased ferritin
Low serum iron levels
Low transferrin saturation
Decreased TIBC
Normocytic, normochromic anemia
(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 435
47
Principal causes of megaloblastic anemia.
Folate deficiency
Vitamin B12 deficiency
(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437
48
Bone marrow is markedly hypercellular as a result of increased number of megaloblasts, which are large cells that have delicate, finely reticulated nuclear chromatin and abundant basophilic cytoplasm.
Megaloblastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437
49
PBS finding in neutrophils and red cells of patients with megaloblastic anemia.
Hypersegmented neutrophils, large, egg-shaped macro-ovalocytes(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 437
50
Difference between megaloblastic and pernicious anemia.
Presence of neurologic abnormalities in pernicious anemia.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
51
Deficiency in folate causes this type of anemia.
Megaloblastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
52
Deficiency in Vitamin B12 absorbtion causes this type of anemia.
Pernicious anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
53
Etiology of pernicious anemia.
1.Vitamin B12 malabsorption secondary to autoantibodies against parietal cells and intrinsic factor which is needed in its absorption.
2. Gastrectomy or ileal resection
(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 438
54
Principal neurologic lesion in pernicious anemia.
Demyelination of posterior and lateral columns of the spinal cord.
(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 439
55
Bone marrow is markedly hypocellular, with >90% of the intertrabecular space being occupied by fat.
Aplastic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 439
56
Tear drop cells are also called __________.
Dacrocytes(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 440
57
Dacrocytes are found in peripheral blood of patients with this type of anemia.
Myelophthisic anemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 440
58
Increase in blood concentration of red cells, with an increase in Hgb concentration.
Polycythemia or erythrocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
59
Polycythemia secondary to reduced plasma volume.
Relative polycythemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
60
Polycythemia secondary to abnormal proliferation of myeloid stem cells and low erythropoeitin levels.
Primary polycythemia (Absolute)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
61
Polycythemia secondary to increased erythropoeitin levels due to lung disease, high-altitude living, cyanotic heart disease and EPO secreting tumors.
Secondary polycythemia (Absolute)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
62
Total white cell count is reduced to 1000 cells/uL. Affected persons are extremely susceptible to bacterial and fungal infections.
Neutropenia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 441
63
A self-limited disease of adolescents and young adults that is causd by B lymphocytotropic EBV characterized by fever, sore throat and generalized lymphadenitis, an increase of atypical lymphocytes in blood and an antibody and T cell response to EBV.
Infectious mononucleosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 442
64
Cells with abundant cytoplasm 12-16um in diameter that contains azurophilic granules, and an oval, indented or folded nucleus.
Atypical lymphocytes (Infectious mononucleosis)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 442
65
Inflamed nodes are swollen, gray-red and engorged. There are large germinal centers containing numerous mitotic figures. Affected nodes are tender and fluctuant if with extensive abscess formation.
Acute nonspecific lymphadenitis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
66
This pattern is associated with infections or inflammatory processes that activate B cells which create a follicular or germinal center reaction. Lymph node architecture is preserved, lymphoid nodules vary in shape and size, mixed lymphocytic infiltrates with prominent phagocytic and mitotic activity within germinal centers.
Follicular hyperplasia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
67
Reactive pattern characterized by distention and prominence of the lymphatic sinusoids, due to marked hypertrophy of lining endothelial cells and an infiltrate of histiocytes. Encountered in lymph nodes draining cancers.
Sinus histiocytosis(TOPNOTCH)
68
Characterized by reactive changes within T-cell regions of the lymph node, usually encountered during viral infections, following certain vaccinations, and immune reactions induced by certain drugs.
Paracortical hyperplasia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
69
Formation of sarcoid-like ganulomas that undergo central necrosis associated with neutrophil accumulation. This irregular stellate necrotizing granuloma is seen in patients presenting with a raised inflammatory node, vesicle or eschar at site of injury. History of exposure to cats.
Cat scratch disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
70
Causative agent for cat scratch disease(TOPNOTCH)
Bartonella henselae(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 444
71
Lymphoblasts with irregular nuclear contours, condensed chromatin , small nucleoli and scant agranular cytoplasm. Blasts compose >25% of marrow cellularity. Most common childhood leukemia.
Acute lymphocytic leukemia (ALL)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 447
72
Frequent small "cleaved" cells mixed with large cells, growth pattern nodular, centroblasts present. Occurs in older adults, usually involves nodes, marrow, spleen. Associated with t(14;18).
Follicular lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 451
73
Small to intermediate-sized irregular lymphocytes growing in a diffuse pattern, no centroblasts and proliferation centers. Occurs mainly in older males, GI tract commonly affected. Associated with t(11;14).
Mantle cell lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 452
74
Plasma cells in sheets, with prominent nucleoli or inclusion containing Ig. Presents as disseminated bone disease, with destructive lytic lesions.
Plasmacytoma / plasma cell myeloma(TOPNOTCH)
75
Intermediate-sized round lymphoid cells with 2-5 prominent nucleoli. Nuclear remnants phagocytosed by interspersed macrophages with abundant clear cytoplasm, "starry sky pattern"
Burkitt lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 453
76
Sheets of small, round lymphocytes and scattered ill-defined foci of larger, actively dividing cells diffusely efface involved LN. A foci of mitotically active cells called proliferation centers are pathognomonic.
Small lymphocytic leukemia (SLL) / Chronic lymphocytic leukemia (CLL)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
77
Fragile neoplastic lymphocytes that are frequently disrupted during smear preparation.
Smudge cells(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
78
Smudge cells are seen in this type of leukemia.
CLL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 450
79
Tumor cells have large nuclei with open chromatin and prominent nucleoli. Most important type of lymphoma in adults, accounting to ~50% of adult NHL.
Diffuse large B-cell lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 452
80
Multifocal destructive bone lesions seen as punched-out defects in bone radiographs.
Multiple myeloma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 455
81
Excess light or heavy chains along with complete Igs synthesized by neoplastic plasma cells.
Bence-Jones proteins(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 454
82
Cells with two mirror-image nuclei or nuclear lobes, each containing a large acidophilic nucleolus surrounded by a distinctive clear zone, imparting an owl-like appearance.
Reed-Sternberg cell(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 456
83
A distinctive groups of neoplasms that arise almost invariably in a single lymph node or chain of lymph nodes and spread characteristically in a stepwise fashion to anatomically contiguous nodes.
Hodgkin Lymphoma(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 456
84
Most common form of Hodgkin lymphoma, characterized by a large cell which has a single multilobate nucleus with small nucleoli and an abundant pale-staining cytoplasm called lacunar cells.
Nodular sclerosis HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 457
85
Most common form of HL in patients greater than 50 years old, with male predominance, plentiful RS cells and heterogenous cellular infiltrates.
Mixed cellularity HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 458
86
Cells found in adult T-cell lymphoma which appear to have multilobulated nuclei.
Cloverleaf or flower cell(TOPNOTCH)
87
Subgroup of HL characterized by a large number of small resting lymphocytes admixed with a variable number of benign histiocytes. Variant RS cells described as multilobed, puffy nucleus Which appears like a "popcorn". Excellent prognosis.
Lymphocyte-predominance HL(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 458
88
Cells with fiery red cytoplasm, seen in Multiple myeloma
Flame cells(TOPNOTCH)
89
Cells with pink globular cytoplasmic inclusions, seen in Multiple myeloma
Russell bodies(TOPNOTCH)
90
Cells with blue globular nuclear inclusions, seen in Multiple myeloma
Dutcher bodies(TOPNOTCH)
91
Multiple nuclei, prominent nucleoli, and cytoplasmic droplets containing Ig.
Bizarre, multinucleated cells(TOPNOTCH)
92
Bizarre multinucleated cells, flame cells, Russel bodies and Dutcher bodies are all seen in what disease?
Multiple myeloma(TOPNOTCH)
93
Bone marrow aspirate shows hypercellular martow packed with myeloblasts and azurophilic needle-like material called Auer rods.
Acute Myelogenous Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 462
94
Leukemia of children most responsive to chemotherapy.
Acute Lymphoblastic Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 461
95
Hodgkin lymphoma subgroup most commonly associated with EBV infection.
Lymphocyte depleted(TOPNOTCH)
96
Mature B-cell tumor in the elderly, where cells have fine hair-like projections (hairy cells).
Hairy cell leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 459
97
t(9;22) is also called ______.
Philadelphia chromosome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 465
98
Hodgkin lymphoma subgroup with highest count of RS cells.
Mixed cellularity type(TOPNOTCH)
99
This correlates with good prognosis in Hodgkin lymphoma.
High Lymphocyte : Reed-Sternberg cell ratio(TOPNOTCH)
100
Hodgin lymphoma subgroup not associated with EBV.
Nodular sclerosis and lymphocyte predominant(TOPNOTCH)
101
Hodgkin lymphoma subgroup with poorest prognosis.
Lymphocyte depleted HL(TOPNOTCH)
102
Tumor of the thymus associated with myastheni gravis and pure red cell aplasia.
Thymoma(TOPNOTCH)
103
Pentalaminar tubules, often with a dilated terminal end (tennis racket-like appearance).
Birbeck granules(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
104
Proliferative disorder of the dendritic cells which has birbeck granules.
Langerhans Cell Histiocytosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
105
Pathology behind polycythemia vera.
Mutation in tyrosine kinase JAK2, which acts in signalling pathways of the erythropoeitin receptors, rendering them hypersensitive to erythropoeitin.(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 466
106
Treatment for polycythemia vera.
Phlebotomy(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 466
107
Collection of aggressive tumors that are comprised of immature myeloblasts which replace the marrow and suppress normal hematopoiesis.
Acute Myelogenous Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
108
Myeloid tumor arising from a pluripotent stem cell associated with mutatios of the BCR-ABL gene. If untreated, may progress to a blast crisis.
Chronic Myelogenous Leukemia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
109
Most common myelodysplastic syndrome. A myeloid tumor in which abnormal megakaryocytes stimulate marrow fibroblasts to release collagen, replacing the marrow space, leading to pancytopenia and extramedullary hematopoeisis.
Myeloid metaplasia with Myelofibrosis(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 467
110
Other name for acute disseminated Langerhans cell histiocytosis.
Letterer-Siwe disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 468
111
Caused by a systemic activation of coagulation pathways, leading to formation of thrombi throughout the microcirculation.
Disseminated intravascular coagulation(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 469
112
Characterized by spontaneous bleeding, prolonged bleeding time, and normal PT and PTT.
Thrombocytopenia(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 471
113
Drug-induced disorder caused by IgG antibodies that bind to platelet factor IV on platelet surfaces, which activates platelets and induce their aggregation.
Heparin-Induced Thrombocytopenia (HIT)(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
114
Associated with pentad of fever, thrombocytopenia, microangipathic hemolytic anemia, transient neurologic deficits and renal failure.
Thrombotic thrombocytic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
115
Associated with childhood onset microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. No neurologic symptoms.
Hemolytic uremic syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
116
Caused by antiplatelet antibodies directed against glycoproteins IIb-IIIa, Ib-IX.
Immune thrombocytopenic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
117
Decreased gp Ib leads to defective platelet adhesion, associated with decreased platelet count.
Bernard-Soulier Syndrome(TOPNOTCH)
118
Caused by deficiency of ADAMTS13, a vWF metalloprotease.
Thrombotic thrombocytopenic purpura(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
119
Caused by shiga-like toxin in EHEC (E. coli O157:H7) from improperly cooked burgers.
Hemolytic uremic syndrome(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 472
120
Decreased gp IIb-IIIa leads to defective platelet aggregation, associated with normal platelet count.
Glanzmann thrombasthenia(TOPNOTCH)
121
Most common bleeding disorder.
vWF disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
122
Most common hereditary disease associated with life threatening bleeding,
Hemophilia A(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
123
An X-linked recessive disorder caused by reduction in factor VIII activity.
Classic Hemophilia/ Hemophilia A(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
124
An X-linked disorder caused by deficiency of Factor IX, or Christmas factor. Bleeding time is normal, PTT is prolonged.
Hemophilia B/ Christmas Disease(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 474
125
A state associated with excessive removal of formed elements of blood, resulting in anemia, leukopenia or thrombocytopenia.
Hypersplenism(TOPNOTCH)Robbins Basic Pathology, 8th Ed. p. 476
126
A 17 y/o male presented with a short history of fever, tonsillitis and monolateral enlarged cervical lymph nodes. PE revealed enlargement of righ cervical lymph node, 3 cm in diameter hard , and pharyngeal hyperemia. Biopsy was done and showed a malignant population of round monomorphic B cells interspersed with macrophages forming the start in the "starry sky" pattern. This is a case of:
Burkitt lymphoma (TOPNOTCH)
127
An 18 y/o male presents with easy fatigability, fever, and cutaneous bleeding. Bone marrow biopsy showed 40% myeloblasts. What is the most likely diagnosis?
AML (TOPNOTCH)
128
The most common cause of agranulocytosis
Drug toxicity (TOPNOTCH) Robbins Pathologic Basis of Disease, 9th ed., p. 582
129
What laboratory finding differentiate leukemoid reaction from CML?
Elevated leukocyte alkaline phosphatase. (TOPNOTCH)
130
An 8 month old presented with anemia. Red cells were noted to be oval and macrocytic. Nutritional history revealed that the infant was exclusively fed raw goat's milk. The most likely cause of his anemia is:
Folate deficiency (TOPNOTCH)
131
A 2 y/o child arrived at well-child clinic. Mother reported that her child is exclusively breastfed for 6 months and then supplemented mainly by carrots. The patient is most likely prone to developing anemia caused by:
Vitamin B12 deficiency (TOPNOTCH)
132
Most common type of cancer in children; highly aggressive tumors manifest with signs and symptoms of bone marrow failure, or as rapidly growing masses
Acute lymphoblastic leukemia/Lymphoblastic lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
133
Most common leukemia of adults which usually presents with bone marrow and lymph node involvement.
Small lymphocytic lymphoma/CLL(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
134
Most common leukemia of adults which usually presents with bone marrow and lymph node involvement.
Follicular lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
135
Most common lymphoma of adults
Diffuse Large B-Cell Lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
136
Very aggressive tumor of mature B cells that usually arise at extranodal sites; strongly associated with translocations involving MYC proto-oncogene
Burkitt Lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 598
137
Plasma cell neoplasm commonly associated with lytic bone lesions, pathologic fractures, chronic pain, hypercalcemia, renal failure, and acquired immune abnormalities.
Multiple Myeloma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 599
138
Major pathologic feature of multiple myeloma
Bone destruction mediated by neoplastic plasma cells(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 599
139
True or False. Cellular immunity is relatively unaffected in Multiple myeloma
True(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 600
140
Single most important factor in the pathogenesis of renal failure in Multiple myeloma
Bence-Jones proteinuria(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 600
141
True or False. Age younger 2 years is associated with a worse prognosis in ALL.
True(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 592
142
Abrupt stormy onset, symptoms of depressed marrow function, and mass effects by neoplastic infiltration , including bone pain are more common in ALL or AML?
ALL (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 592
143
The leading cause of cancer deaths in children
ALL (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 592
144
This lymphoma often presents a mass involving the mandible and shows an unusual predilection for involvement of abdominal viscera, particularly the kidneys, ovaries, and adrenal glands.
Endemic Burkitt lymphoma(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 597
145
A 40 y/o male presented with unexplained weight loss, fever, and night sweats. Chest radiograph showed a mediastinal mass. Histologic findings showed large cells with multiple nuclei with large inclusion-like nucleolus. What is the most likely diagnosis?
Hodgkin Lymphoma (Presence of Reed-Sternberg cells) (TOPNOTCH)
146
A 52 y/o female presents with dragging sensation in the abdomen associated with anemia, weakness, and weight loss. Chromosomal analysis showed presence of BCR-ABL fusion gene. What is the most likely diagnosis?
CML (TOPNOTCH)
147
Presence of anemia, hemoglobinemia, hemoglobinuria, hemosiderinuria, and jaundice are manifestations of intravascular or extravascular hemolysis?
Intravascular hemolysis(TOPNOTCH)Robbins Basic Pathology, 9th ed., p. 631
148
Patient presents with anemia, splenomegaly, and jaundice. Peripheral blood smear shows small, dark-staining red cells lacking the central zone of pallor. Father had prolonged jaundice since childhood. What is the most likely diagnosis?
Hereditary spherocytosis(TOPNOTCH)
149
A 5 y/o male presented with malaise and low grade fever for 10 days. He was diagnosed with toxoplasmosis and was given pyrimethamine and sulfadiazine. Three days after, patient presented with jaundice and dark urine. PBS showed Heinz bodies and bite cells. What is the most likely diagnosis?
G6PD Deficiency(TOPNOTCH)
150
Hemolysis and vaso-occlusive crisis are common in this form of anemia caused by mutaion of glutamic acid to valine.
Sickle-cell anemia(TOPNOTCH)
151
The most common trigger for episodic hemolysis in G6PD Deficiency
Infection(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 634
152
A 19 y/o African-American male presented with severe pain in the chest and extremities, splenomegaly, and anemia. PBS showed reticulocytosis, presence of target cells, and sickled cells. Three days prior , patient developed cough and fever. For the past 3 years, patient had suffered from recurrent pains and jaundice. What is the pathophysiologic mechanism responsible for the most serious clinical features of this disease?
Microvascular occlusion (in Sickle cell disease) (TOPNOTCH)
153
Leading cause of disease-related death in individuals with PNH
Thrombosis(TOPNOTCH) Robbins Basic Pathology, 9th ed. P. 642
154
The immediate cause of megaloblastosis, and a common denominator of folic acid and vitamin B12 deficiency
Suppressed synthesis of DNA (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 648
155
Most common nutritional disorder in the world
Iron Deficiency Anemia(TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 649
156
An 18 y/o female complained of generalized weakness, lethargy, and light headedness. She revealed she was having excessive bleeding during menstruation from the previous 6 months. Upon examining, she was noted to have pallor, tachycardia, and swollen tongue. Most likely morphology of RBC:
Microcytic, hypochromic anemia (Case of IDA) (TOPNOTCH)
157
Presents signs and symptoms of anemia, thrombocytopenia, and neutropenia. Splenomegaly is characteristically absent. And the red cell are usually macrocytic and normochromic. (+) Reticulocytopenia. Bone marrow is hypocellular. What is the condition described?
Aplastic anemia(TOPNOTCH)Robbins Basic Pathology, 9th ed. p. 654
158
Virus implicated in acute red cell aplasia
Parvovirus B19 (TOPNOTCH)) Robbins Basic Pathology, 9th ed., p. 655
159
Most feared complication of thrombocytopenia
Intracranial bleeding (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 657
160
Most common presenting symptoms of this condition are spontaneous bleeding from mucous membranes, excessive bleeding from wounds, or menorrhagia. Bleeding tendency often goes unnoticed until some hemostatic stress, such as surgery, reveals its presence. Patients may have defects in platelet function despite a normal platelet count, prolonged PTT.
von Willebrand disease (TOPNOTCH) Robbins Basic Pathology, 9th ed., p. 662
161
Patient with this disease has a tendency toward easy bruising and massive hemorrhage after trauma or operative procedures, and recurrent bleeding into the joints. Petechiae are absent. Patients have a prolonged PTT and normal PT.
Hemophilia A and B (Factor VIII and IX Ddeficiency) (TOPNOTCH) Robbins Basic Pathology, 9th ed. p. 663
162
Two major mechanisms that trigger DIC
Release of tissue factor and widespread endothelial injury(TOPNOTCH) Robbins Basic Pathology, 9th ed. P. 663
163
A 5 year old male is observed by his mother to have tea-colored urine whenever he has infections. One particular episode of pneumonia required hospital admission, where the child developed jaundice also. A peripheral blood smear showed red cells with Heinz bodies, while other red cells had parts of their cytoplasm "plucked out". The child likely has a deficient enzyme required in the production of reduced glutathione, the gene for which is found on (A) X chromosome (B) Y chromosome (C) chromosome 21 (D) chromosome 22
X chromosome (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 431-432
164
An 8 year old child from the slums has easy fatigability and pallor. A peripheral blood smear shows large, egg-shaped red cell precursors, and hypersegmented neutrophils. Short of measuring serum folate and vitamin B12 levels, what finding will suggest a vitamin B12 deficiency, rather than a folate deficiency? (A) gastrointestinal symptoms (B) neurologic symptoms (C) development of congestive heart failure (D) megaloblasts on bone marrow aspirate smears
neurologic symptoms (TOPNOTCH)Robbins Basic Pathology, 8th Ed. Pp 437-439
165
A 33 year old female presents with pallor, easy fatigability, and echymoses. Her spleen is not enlarged. A CBC showed profound anemia and markedly decreased WBC and platelet counts. A bone marrow core biopsy showed marrow that is predominantly replaced by fat, with few lymphocytic and plasma cells. The most common cause of her condition is (A) myelotoxic drugs (B) viral infection (C) bacterial infection (D) idiopathic
idiopathic (aplastic anemia) (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P439
166
A 14 year old male develops fever, sore throat, lymphadenitis, and fatigue. His CBC shows leukocytosis, with a lymphocytic predominance. Peripheral blood smear shows some large leukocytes with abundant cytoplasm occasional azurophilic granules, and indented nuclei with fine chromatin. Monospot test and anti-EBV titers are positive. The large leukocytes seen are (A) monocytes (B) megakaryocytes (C) B cells (D) T cells
T cells (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 443
167
Lymph nodes that drain cancers but do not yet harbor metastatic deposits often show (A) follicular hyperplasia (B) paracortical hyperplasia (C) sinus histiocytosis (D) fibrous obliteration
sinus histiocytosis (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 444
168
A 55 year old male presents with pallor and easy fatigability. Physical exam showed massive splenomegaly. CBC showed marked leukocytosis, and a peripheral smear showed numerous neutrophils, metamyelocytes, and myelocytes. Basophils are also seen. A bone marrow aspirate shows a similar picture. This man likely has (A) a BCR-ABL fusion gene (B) a JAK2 mutation (C) an 8:22 translocation (D) an 11:22 translocation
a BCR-ABL fusion gene (Chronic myelogenous leukemia) (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 464
169
In primary myelofibrosis, marrow fibroblasts are stimulated to proliferate by PDGF and TGF-beta released from (A) granulocyte precursors (B) erythroid precursors (C) lymphoid cells (D) neoplastic megakaryocytes
neoplastic megakaryocytes (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 466
170
A 28 year old female is found to have a mediastinal mass on chest xray during a preemployment medical exam. On history, she is found to have muscle fatigue that worsens as the day progresses. She undergoes surgery where her mediastinal mass is resected. Microscopic examination showed sheets of bland spindle cells with sparse inflammatory infiltrates. Her tumor is (A) a metastasis from an undiagnosed endometrial mass (B) a metastasis from an occult breast malignancy (C) a lymphoma(D) a thymoma
a thymoma (TOPNOTCH)Robbins Basic Pathology, 8th Ed. P 476
171
AML - Acute Myeloid Leukemia
ALL - Acute Lymphoblastic Leukemia
CL - Chronic Leukemia
MPD - MYELOPROLIFERATIVE DISORDERS
- PV - Policitemia Vera
- CML - Chronic Myeloid Leukemia
- ET - Essential Trombocytemia
172
Imagen de nodulo linfático y que regiones estarán agrandadas en caso de diferentes enfermedades
Corteza - Linf. B - Artritis Reumatoide y HIV
Paracorteza - Linf. T - Infecciones virales (Epstein Barr)
Medula - drenage de una region del cuero que tenga cancer
173
Cuales son los tejidos linfoides?
Timo
Ganglio linfático
Bazo
174
MORFOLOGIA MICRO DE UN GANGLIO
LINFATICO:
Tinción H&E o giemsa
* Corterza
* Zona paracortical/ interfolicular
* Zona medular
* Sistema sinusoidal
* Capsula y tejido adyacente
175
INMUNOHISTOQUIMICA
Marcadores utilizados rutinariamente:
**CD45**: celula inmadura
**Linfocitos B**: **CD19**, **CD20**, CD79a, KiB5,
VS38c, cadenas livianas, CD23, **CD10**, Bcl6,
Ciclina D1.
**Linfocitos T**: **CD3**, CD43, CD45RO, **CD2**,
**CD7**, **CD4/8**.
**Macrófagos**: CD68
Otros: **Bcl2, CD15, CD30**, ALK-1, MUM-1, MPX, CD34, CD1a, Pax 5, etc.
176
Ganglio linfatico normal
177
Importante al resecar un ganglio:
- Evitar biopsias inguinales
- Elegir cervicales y supraclaviculares
- En caso de existir un grupo de ganglios
seleccionar el mayor
- Resecarlo completo
178
Cuales son los tipos de neoplasias LINFOIDES?
o Neoplasias de **precursores** de Linfocitos B
o Neoplasia de Linfocitos B **periféricos**
o Neoplasias de **precursores** de Linfocitos T
o Neoplasias de Linfocitos T y NK **periféricos**
o Linfoma de **Hodgkin**
179
NEOPLASIA DE PRECURSORES DE LINFOCITOS B
Leucemia/linfoma linfoblástico agudo de linfocitos B (LLA-B)
180
NEOPLASIAS DE LINFOCITOS B PERIFERICOS:
- Leucemia linfocítica crónica/linfoma linfocitico pequeño
- Leucemia prolinfocítica de linfocitos B
- Linfoma linfoplasmocítico
- Linfomas esplénicos y ganglionares de la zona marginal
- Linfoma de las células del manto
- Linfoma folicular
- Linfoma de la zona marginal
- Tricoleucemia
- Plasmocitoma mieloma de células plasmáticas
- Linfoma difuso de linfocitos B grandes
- Linfoma de Burkitt
181
NEOPLASIA DE PRECURSORES DE LINFOCITOS T:
Leucemia/linfoma linfoblástico agudo de
infocitos T
182
NEOPLASIAS DE LINFOCITOS T Y LINFOCITOS CITOLÍTICOS NATURALES PERIFERICOS
- Leucemia prolinfocítica de linfocitos T
- Leucemia linfocítica de gránulos grandes
- Micosis fungoide/Sindrome de Sézary
- Linfoma anaplásico de células grandes
- Linfoma angioinmunoblástico de linfocitos T
- Linfoma de linfocitos T asociado a enteropatía
- Linfoma de linfocitos T similar a paniculitis
- Linfoma de linfocitos T hepatoesplénico
- Leucemia/linfoma de linfocitos T del adulto
- Linfoma de linfocitos citolíticos naturales/T extraganglionar
- Leucemia de linfocitos citolíticos naturales
183
# Linfoma de Hodgkin
Cuales son los subtipos clasicos?
* Esclerosis nodular
* Celularidad mixta
* Rico en linfocitos
* Depleción linfocítica
NO CLASICO: **Predominio linfocitico nodular**
183
# Linfoma de Hodgkin
Cuales son los subtipos clasicos?
* Esclerosis nodular
* Celularidad mixta
* Rico en linfocitos
* Depleción linfocítica
**Predominio linfocitico nodular**
184
Nacen de la medula osea, se diferencian en el ganglio, termina su maduración y se transforman en células plasmáticas.
Linfoma B
185
# FACTORES ETIOLÓGICOS Y PATOGÉNICOS
Translocaciones cromosómicas y otras
mutaciones adquiridas:
* MALT1
* BCI10
* BCL6
* C-MYC
186
Factores genéticos hereditarios:
* Síndrome de Bloom, anemia de Fanconi ↑ leucemia en adultos
* Síndrome de Down y NF1 ↑ leucemia en infancia
187
Que virus están involucrados con diferentes tipos de enfermedades del sist. linfatico?
- HTLV-1 (virus 1 de la leucemia de linfocitos T humana) LEUCEMIA/LINFOMA DE LINFOCITOS T DEL ADULTO
- VEB LINFOMA DE BURKITT Y Linfoma de Hodgkin
- KSHV/HHV-8 (herpes virus de sarcoma de Kaposi/herpes humano 8)
188
# FACTORES ETIOLÓGICOS Y PATOGÉNICOS
Estimulación inmunitaria crónica:
El H. pylori y HIV están relacionados a que tipos de linfomas?
- H. pylori - LINFOMA B
- HIV - LINFOMAS B ASOCIADOS A VEB
189
Neoplasias que se presentan con afectación extensa y normalmente de la **MO**, pero puede presentar en la sangre periférica. **No se presenta como masa**, pues afecta la MO.
Leucemia
190
Proliferaciones que surgen como **masas tisulares** **delimitadas**. Mucho mas frecuente **en ganglios**, con característica predominante de una **masa**. Existen algunos tipos que pueden leucemizarse al afectar la MO.
Linfoma
191
2/3 de los LNH (linfoma no hodgkin) y
prácticamente todos los LH (linfoma hodgkin) se presentan con **........** y **...........**.
2/3 de los LNH (linfoma no hodgkin) y
prácticamente todos los LH (linfoma hodgkin) se presentan con **adenopatías** y **adenomegalia**.
192
LEUCEMIA/LINFOMA LINFOBLASTICOS AGUGOS
Son neoplasias compuestas por células **...........** B (pre-B) o T (pre-T) que se consideran linfoblastos.
LEUCEMIA/LINFOMA LINFOBLASTICOS AGUGOS
Son neoplasias compuestas por células **inmaduras** B (pre-B) o T (pre-T) que se consideran linfoblastos.
193
o 85%
o Se manifiestan como “leucemias” agudas
o Infancia (3 años)
LLA-B
194
o menos frecuentes
o Se manifiestan como “linfomas” del timo
o Varones adolescentes
*es la neoplasia mas fr en niños (< 15 anos)
LLA-T
195
INMUNOMARCACION
- TdT + 95 %
- LLA-B:
- LLA-T:
- LLA-B: CD19, PAX5, CD10
- LLA-T: CD1, Cd2, CD5, Cd7
196
Dolores en las articulaciones sin infecciones en jóvenes siempre sospechar de:
Leucemia
197
Clínica de las leucemias agudas
o Inicio brusco “tormentoso” DÍAS O SEMANAS
o Síntomas por depresión de lafunción medular:
Anemia
Fiebre por infecciones
Hemorragias
o Efectos de masa
Dolor óseo
Linfadenopatías
Esplenomegalia
o Manifestaciones en el SN
Cefalea
Vómitos
198
Leucemias agudas. Pronóstico
o Con QT 95% de los niños remisión completa, 75-85% se cura
o Con QT solo el 35-40% de los adultos se cura
199
Es la leucemia más frecuente en adultos
Edad media: 60 años, V:M 2:1
linfocitos > 5000/mm3
LEUCEMIA LINFOCÍTICA CRÓNICA (LLC)
200
* Neoplasias de los linfocitos B periféricos
* Dos trastornos que difieren solo en el grado de linfocitosis en sangre periférica.
LEUCEMIA LINFOCÍTICA CRÓNICA (LLC)
LINFOMA LINFOCITICO PEQUEÑO (LIP)
201
Que son las sombras de Gumprecht?
Son núcleos desnudos, como resultado de la destrucción de linfocitos, que por su fragilidad intrínseca adoptan un aspecto amorfo y desestructurado, al realizarse la extensión de sangre.
Las sombras de Gümprecht en los frotis de sangre periférica pueden ser indicadores de la presencia de patologías malignas como la leucemia linfocítica crónica.
202
LEUCEMIA LINFOCITICA CRÓNICA (LLC)
Que encontramos en sangre periferica, MO, bazo e hígado?
- Sangre periférica: linfocitosis
- MO: infiltrados intersticiales o agregados tumorales
- Bazo: infiltración en pulpa blanca y roja
- Higado: infiltración en tractos portales
203
LEUCEMIA LINFOCÍTICA CRÓNICA (LLC)/
LINFOMA LINFOCITICO PEQUEÑO (LLP)
Clínica
- Asintomática
- Síntomas inespecíficos (cansancio, perdida de peso y anorexia)
- 50% Linfadenopatías y hepatoesplenomegalia
- Se asocia a anemia hemolítica autoinmune y trombocitopenia inmune
- Tendencia a transformarse a Linfoma difuso de grandes células B (Síndrome de Richter).
- **Mal pronóstico.**
204
Cuale es el linfoma NO Hodgkin INDOLENTE más frecuente?
LINFOMA FOLICULAR
205
- Ambos sexos
- Edad mediana
- Surge de linfocitos B del centro germinal
- Se asocia a translocaciones cromosómicas que afectan al gen **BCL-2**
- BAZO: pulpa blanca
- HIGADO: triadas portales
Que linfoma es?
LINFOMA FOLICULAR
- LNH indolente más frecuente
206
Nódulos prominentes que representan folículos de pulpa blanca
LINFOMA FOLICULAR
DD:
- LLP: linfoma de linfocitos pequeños;
- LCM: linfoma células del manto;
- LZM: linfoma de la zona marginal
207
LINFOMA FOLICULAR
- Crecimiento nodular o nodular y difuso
- Células pequeñas con núcleos irregulares o hendidos y citoplasmas escasos (células hendidas pequeñas). Son la mayoría
- Células mayores con cromatina nuclear abierta, varios nucléolos, citoplasmas moderados (centroblastos)
208
LINFOMA FOLICULAR
Cual es la traslocación que determina esta enfermedad?
t[14;18]
INMUNO: CD19, CD20, CD10, BCL-6, BCL-2 (90%)
209
LINFOMA FOLICULAR
Clínica
- Linfadenopatías indoloras generalizadas
- Tubo digestivo
- SNC
- Testículos
- Evolución con recaídas y mejorías
- Supervivencia mediana 7-9 años
210
- Es la forma mas frecuente de LNH (Linfoma NO de Hodgkin)
- Mortal sin tratamiento
- Hombres, 60 años
LINFOMA DIFUSO DE LINFOCITOS B GRANDES
211
LINFOMA DIFUSO DE LINFOCITOS B GRANDES
Clínica
- **Masa tumoral destructiva**, grande ganglionar o extra-ganglionar
- Puede afectar: **Anillo de Waldeyer**, amígdalas, adenoides, hígado, bazo, tubo digestivo, piel, hueso, cerebro
- MO infrecuente, tardía
212
Micro:
LINFOMA DIFUSO DE LINFOCITOS B GRANDES
Infiltración difusa por células grandes con núcleos grandes multilobulados, nucléolos múltiples, citoplasma abundante
INMUNO: CD19, CD20, CD10, BLC-6
213
- 30% LNH en la infancia
- Niños y adultos jóvenes
- Muy agresivo
- 3 subtipos “histológicamente idénticos”:
**AFRICANO O ENDEMICO** *relacionado al virus Epstein-barr, afecta la **mandibula** con **grandes masas tumorales**
ESPORADICO
ASOCIADO A HIV
- Difieren en la clínica, genotipo y virología. - > localizaciones extraganglionares
LINFOMA DE BURKITT
214
LINFOMA DE BURKITT
Que se ve en la micro?
- Macrófagos (“**patrón en cielo estrellado**”)
- Infiltrado difuso de células linfoides de tamaño intermedio con núcleos redondeados, cromatina grosera, varios nucléolos y moderado citoplasma
- Elevado índice mitótico
- Numerosas células apoptóticas
215
LINFOMA DE BURKITT
Expresa que tipos de "CD"? Que gen es traslocado?
- CD19, CD20, CD10, BCL-6 + (LB centro germinal)
- **NO** expresa **BCL-2**
- Translocación del gen **c-MYC** (crom 8)
216
Poco frecuente
< 10% de los LNH
50-60 años, varones
Mal pronóstico
LINFOMA DE LAS CELULAS DEL MANTO
217
LINFOMA DE LAS CELULAS DEL MANTO
Micro:
- Las células tumorales se parecen a los LB normales de la ZM que rodean los CG.
- Linfocitos pequeños con núcleos irregulares, a veces hendidos, citoplasma escaso, nucléolos débil, cromatina condensada.
218
- Linfadenopatías indoloras generalizadas
- Afectación del Sistema Pulmonar 20-40% de los casos
- Afectación esplénica (50%) e intestinal (poliposis colónica)
- Agresivo, sobrevida 3-4 años dependiendo de diferentes factores
- Trasplante de MO
LINFOMA DE LAS CELULAS DEL MANTO
219
LINFOMA DE LAS CELULAS DEL MANTO
Inmuno:
CD19, CD20, CD 5, **Ciclina D1**+
CD23 -
220
Inicialmente se describió en mucosas - linfomas MALT (tejido linfoide asociado a mucosas).
Surgen de tejidos afectados por trastornos inflamatorios crónicos de etiología autoinmune o infecciosa.
- Glándula salival en la **Enf. de Sjögren**,
- Tiroides en la **tiroiditis de Hashimoto**
- Estomago en la **gastritis por H. pylori**.
LINFOMA DE LA ZONA MARGINAL
Se mantienen localizados durante periodos de tiempo prolongados, diseminándose sistémicamente en etapas avanzadas.
Puede haber remisión si se erradica el agente causal.
221
LINFOMA DE LA ZONA MARGINAL
MICRO:
- linfocitos B pequeños (patrón de infiltración)
- Presencia de folículos
- Lesiones intraepiteliales
222
LINFOMA DE LA ZONA MARGINAL
LOCALIZACIONES + fr:
- Estomago
- Cabeza y cuello
- Ojo
- Piel
- pulmón
223
- Proliferaciones de linfocitos B que producen/liberan Ig monoclonal
- La más frecuente es el **mieloma múltiple** (MM)
NEOPLASIA DE CÉLULAS PLASMATICAS
224
- Sintetizan una cantidad excesiva de cadenas ligeras o pesadas junto a las Ig completas, a la cual se denomina **Componente M**.
- Las cadenas ligeras libres son pequeñas y se eliminan por orina (**proteína de Bence Jones**)
NEOPLASIA DE CÉLULAS PLASMATICAS
225
NEOPLASIA DE CÉLULAS PLASMATICAS
Entidades clínico patológicas:
- **Mieloma Múltiple** - plasmocitoma
- Macroglobulinemia de Waldenström - IgM
- Enfermedad de cadenas pesadas
- **Amiloidosis primaria**
- Gammapatía monoclonal de significado incierto (GMSI)
226
- Es la gammapatía monoclonal más importante
- Proteína M >3g/dl + compromiso MO >10% células plasmáticas
- 65 años
MIELOMA MULTIPLE
227
MIELOMA MULTIPLE
- **Masas tumorales** de células plasmáticas destructivas dispersas por todo el **esqueleto** (fracturas patológicas, dolor crónico)
- Producción excesiva de **Ig**
- Supresión de la inmunidad humoral normal (infecciones bacterianas recurrentes)
- Anemia, **lesiones oseas, hipercalcemia**, fallo renal
228
MIELOMA MULTIPLE
Huesos mas afectados:
* Columna vertebral,
* costillas,
* cráneo,
* pelvis,
* fémur,
* clavícula
* escapula
229
Rx: Defectos en saca bocados (1.4 cm.)
MO: plasmocitos 30% de la celularidad (10% celularidad + CM)
SP: Eritrocitos en **pilas de monedas** (característico, no especifico)
MIELOMA MULTIPLE
230
MIELOMA MULTIPLE
- Pronóstico variable
- Frecuente recaídas
- Tratamiento inmunomodulador, inhibidores proteosoma y Trasplante MO
231
Variante MM quiesciente (smoldering)
- Proteína M >3g/dl, infiltrado MO >10% células plasmáticas pero SIN síntomas
- Evolucionan a MM
232
- Variante infrecuente que se presenta como una masa aislada en hueso o partes blandas
PLASMOCITOMA (MIELOMA SOLITARIO)
233
El plasmocitoma óseo solitario evoluciona
casi inevitablemente a un ........ ..........
Mieloma Multiple
234
- Lesiones extraóseas: Pulmones,
orofaringe, senos paranasales
PLASMOCITOMA (MIELOMA SOLITARIO)
235
- Síndrome en donde los niveles altos de IgM causan síntomas por hiperviscosidad sanguínea
- Adultos mayores
MACROGLOBULINEMIA DE WALDENSTROM
236
- Rara
- Asociada a linfoma linfoplasmocitario
- Síntesis y secreción de los fragmentos libres de cadenas pesadas.
ENFERMEDAD DE CADENAS PESADAS
237
Ocurre como consecuencia de una proliferación monoclonal de células plasmáticas que segregan cadenas ligeras que se depositan como amiloide
Amiloidosis primaria
238
- Sin síntomas que tienen componentes M en sangre (<3g/dl), ancianos, 1% anual se transforma a MM
- Asociada a otras entidades autoinmunes y linfoproliferativas
Gammapatía monoclonal de significado indeterminado (GMSI)
239
- Mal pronóstico
- CLINICA: linfadenopatías generalizadas, eosinofilia, prurito, fiebre, pérdida de peso
MICRO:
- Ninguna característica morfológica patognomónica
- Borramiento difuso del GC por parte de linfocitos T pleomórficos de tamaño variable
- Eosinófilos y macrófagos (reactivos)
- Neoangiogenia
INMUNO: **CD2, CD3, CD5** +, CD19 -, CD 25 -
LINFOMA DE LINFOCITOS T PERIFÉRICOS
240
- Poco frecuente
- Se define por la presencia de reordenamientos del gen ALK en el crom 2p23 (actividad tirosina quinasa que activa vía JAK-STAT)
- Pronóstico bueno (curación con QT: 80%)
CLINICA:
- Niños y adultos jóvenes
- Afectación extraganglionar (60%)
LINFOMA ANAPLASICO DE CELULAS GRANDES
241
- Células anaplásicas grandes con núcleo en herradura y citoplasma voluminoso (hallmark cell)
- Fondo inflamatorio
- Crecimiento paracortical o sinusoidal
- Afectando vénulas
- Necrosis
- INMUNO: ALK, CD30 +
LINFOMA ANAPLASICO DE CELULAS GRANDES
242
- Asociado a infección por **HTLV-1**
- Endémico en Japón, Africa occidental y Caribe
CLÍNICA: Linfoadenopatías generalizadas, hepatoesplenomegalia, linfocitosis en sistema pulmonar, hipercalcemia y lesiones cutáneas
Rápidamente progresiva y mortal
LEUCEMIA / LINFOMA T DEL ADULTO
243
Proliferación de linfocitos T CD4+ cooperadores en piel
3 etapas: Premicótica inflamatoria, en placas y tumoral
Progresión a afectación ganglionar y de MO
MICOSIS FUNGOIDE / SME DE SEZARY
244
MICOSIS FUNGOIDE / SME DE SEZARY
Sme de Sézary: eritrodermia generalizada y linfocitos T circulantes en sistema pulmonar
245
* Surge en un único ganglio o cadena ganglionar
* Se disemina primero hacia los tejidos linfoides continuos anatómicamente
LINFOMA DE HODGKIN
246
LINFOMA DE HODGKIN
Características morfológicas distintivas:
- Células de **Reed Sternberg**
- Liberan factores que inducen la acumulación de linfocitos, macrófagos y granulocitos reactivos (representan el 90% de la celularidad tumoral)
247
LINFOMA DE HODGKIN
Clínica:
* Más frecuente en adultos jóvenes y adolescentes
* Es curable en la mayoría de los casos (80% remisión completa)
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LH: clasificación
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Células de Reed Sternberg
- Variante clásica: **CD15+, CD30** +, CD20-
- Su **identificación es esencial para el diagnostico**.
- Son **células grandes con múltiples núcleos** o un solo núcleo con múltiples lóbulos nucleares, cada uno con un gran nucléolo a modo de inclusión con el tamaño aprox. de un linfocito pequeño.
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- Forma mas frecuente (70%) de LH (Linfoma de Hodgkin).
- Adolescentes y adultos **jóvenes**. Sexo **femenino**
- GL mas afectados: cervicales, supraclaviculares y mediastínicos
- **Pronostico excelente**
- Afectación EG (extraganglionar): rara
LH ESCLEROSIS NODULAR
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LH ESCLEROSIS NODULAR
- célula de RS **variante lacunar** sobre un fondo maduro con linfocito, eosinófilos, plasmocitos y macrófagos.
- deposito de colágeno en blandas que dividen el ganglio en nódulos circunscriptos
- Fibrosis
- (+) para PAX5, CD15, y CD30
- (-) para CD45
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- 20-25%
- > hombres, incidencia bifásica
- Se presenta en estadio III o IV
- Pronostico global es muy bueno
- Relación con VEB
LH CELULARIDAD MIXTA
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LH CELULARIDAD MIXTA
Borramiento difuso de la arquitectura por un infiltrado células heterogéneo que contiene linfocitos T, eosinófilos, plasmocitos y macrófagos benignos, mezclados con CRS clásicas y mononucleares
Las CRS están infectadas por VEB (79%)
IHQ:
- (+) para PAX5, CD15, y CD30
- (-) para CD45
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- Infrecuente
- Más frecuente en varones
- Adultos mayores
- VEB 40%
- Pronóstico muy bueno o excelente
LH RICO EN LINFOCITOS
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- Linfocitos reactivos suponen la inmensa mayoría del infiltrado celular
- Afectación difusa
- CRS variante mononuclear
LH RICO EN LINFOCITOS
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- LH menos frecuente (‹5%)
- Ancianos, VIH +
- Evolución menos favorable (estadios avanzados y síntomas sistémicos)
LH CON DEPLECIÓN LINFOCÍTICA
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LH CON DEPLECIÓN LINFOCITICA
MICRO: Escases de linfocitos y abundancia relativa de Células Reed Steinberg o variantes
INMUNO: idéntico al LH EN
- DD: LNH de células grandes
VEB 90%
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- Variante “**no clásica**”
- 5%
- > hombres jóvenes
- GL (ganglios linfaticos) cervicales o axilares
- Afectación mediastinal y de MO rara
- Pronostico excelente
- **No se asocia a VEB**
LH DE PREDOMINIO LINFOCITICO NODULAR
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- infiltrado nodular de linfocitos pequeños mezclados con macrófagos
- CRS variante LH (linfocítica e histiocítica) que tiene un núcleos multilobulado que se semeja a una palomita de maíz
- **CRS (celulas de reed steinberg) clásicas ausentes**
- Eosinófilos y plasmocitos escasos o ausentes
Células RS expresan marcadores típicos de los linfocitos B del centro germinal (CD20 y BCL-6), **negativo para CD15 y CD30.**
LH DE PREDOMINIO LINFOCITICO NODULAR
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DIFERENCIAS LH Y LNH
**Linfoma de Hodgkin**
- Localizado en **un solo grupo ganglionar** axial (cervical, mediastínico, paraaórtico)
- Dispersión ordenada por contigüidad
- Ganglios **mesentéricos** y anillo de **Waldeyer** **raramente afectados**
- Presentación **extraganglionar infrecuente**
**Linfoma NO de Hodgkin**
- Afectación mas frecuente de **múltiples ganglios periféricos**
- Se disemina, pero **no por contigüidad**
- Afectación **frec del anillo de Waldeyer y
ganglios mesentéricos**
- Presentación **extraganglionar habitual**
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NEOPLASIAS MIELOIDES
Se originan de la célula hematopoyética progenitora mieloide.
Tres categorías:
- Leucemia mieloide aguda
- Síndromes mielodisplásicos
- Trastornos mieloproliferativos
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- Tumor de progenitores hematopoyéticos causado por mutaciones oncogénicas que provoca **acumulación de blastos mieloides** **en MO**.
- Conduce a fracaso medular
- 60 años o más
LEUCEMIA MIELOIDE AGUDA (LMA)
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LEUCEMIA MIELOIDE AGUDA (LMA)
Diagnóstico
- Presencia de al menos **20% de blastos en MO**
- Mieloblastos o monoblastos
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CLASIFICACIÓN MORFOLOGICA LMA
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LEUCEMIA MIELOIDE AGUDA (LMA)
Citogenética:
- Se correlacionan con el cuadro clínico. En jóvenes t[8;21] , inv(16) y t[15;17].
- En adultos mayores generalmente es secundaria a Smes mielodisplásicos (del o monosomias cromosoma 5 y 7).
MOLECULAR
- CBF1α y CBF1β
- PML-RARα
- FLT3
- C-KIT
- NPM
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Enfermedad clonal del stem cell, con displasia y hematopoyesis inefectiva de 1 o más líneas. Frecuente evolución a LMA (leucemia mieloide aguda).
En >70 años.
SINDROMES MIELODISPLASICOS
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ETIOPATOGENIA
- Primaria (de novo)
- Quimioterapia – Radioterapia – Benceno
- Enfermedades hereditarias
CLÍNICA
- Síndrome anémico
- Petequias - hematomas
- Organomegalias - adenopatías (infrecuentes)
- Anemia, trombocitopenia, leucopenia. Reticulocitos normales o ↑
- ↑ ácido úrico y LDH.
SINDROMES MIELODISPLASICOS
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SINDROMES MIELODISPLASICOS
Diagnóstico
- **Citopenia > 6 meses en al menos 1 linaje celular** y exclusión de otra causa de citopenia.
- Displasia morfológica en > 10% células MO o anomalía CTG o 5-19% blastos.
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Son trastornos que presentan tirosina quinasas constitutivamente activadas que evitan los controles normales y producen proliferación y sobrevida de los progenitores de manera independiente.
TRASTORNOS MIELOPROLIFERATIVOS (NMPC)
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TRASTORNOS MIELOPROLIFERATIVOS (NMPC)
NMPC: neoplasias mieloproliferativas crónicas
Cuales son?
- Leucemia mieloide crónica
- Policitemia Vera
- Trombocitemia esencial
- Mielofibrosis primaria
- Leucemia neutrofílica crónica
- Leucemia eosinofílica crónica
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* Proliferación células pluripotenciales y granulocitos con presencia de **cromosoma Philadelphia** t[9:22] o BCR/ABL
* 15% de las leucemias (3% en infancia)
* Edad 65. A > edad > mortalidad
LEUCEMIA MIELOIDE CRÓNICA (LMC)
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LEUCEMIA MIELOIDE CRÓNICA (LMC)
Hipercelular, hiperplasia granulocítica
Citogenético t[9;22] - Molecular BCR/ABL+
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LEUCEMIA MIELOIDE CRÓNICA (LMC)
Clínica:
- Fatiga – intolerancia al ejercicio
- Sudoración - Disminución de peso
- Anorexia – Saciedad precoz
- Palidez
- Esplenomegalia (90% casos)
- Hepatomegalia (10-15%)
- Anemia NN – eritroblastos en SP
- Leucocitosis (>25.000/mm3) Neutrofilia – basofilia
- Trombocitosis (50%)
- Hiperuricemia - LDH elevada (80%)
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- Proliferación clonal de células hematopoyéticas a **predominio serie eritroide**
- Edad 60 años
- Predominio masculino
POLICITEMIA VERA (PV)
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ETIOPATOGENIA: Mutación V617F del gen **JAK-2** (>90%)
POLICITEMIA VERA (PV)
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POLICITEMIA VERA (PV)
Clínica:
- Astenia – disminución de peso
- Sudoración – Prurito acuógeno - Eritromelalgia – facie pletórica
- Trombosis venosa - arterial *TVP- Budd-Chiari – porta - esplénica
- Sme de **hiperviscosidad**
- Hepatomegalia (15%) Esplenomegalia (50%)
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LABORATORIO
- Hb >16,5 o 18,5g/dl
- Leucocitosis con neutrofilia – Trombocitosis
- Aumento uricemia, LDH
- EPO normal
POLICITEMIA VERA (PV)
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* Trastorno clonal de progenitores con **hiperplasia megacariocítica**
* Igual ambos sexos
* Edad 50-60 años
* En <40 años es más frec en mujeres
* Asintomática en 50% de los casos
TROMBOCITEMIA ESENCIAL (TE)
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TROMBOCITEMIA ESENCIAL (TE)
Clínica
- Eritromelalgia – isquemia digital – cianosis
- Trombosis arterial (+frec) y venosa
- **Hemorragias (>1.500.000 plaquetas)**
- Esplenomegalia moderada (10%)
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* Mutación **JAK-2** + (50%)
* Agregación plaquetaria **anormal**
TROMBOCITEMIA ESENCIAL (TE)
Sobrevida no difiere de la población general Evoluciona a:
- LMA espontánea <1%
- LMA asociada a tto 3-10%
- Mielofibrosis 3-10% a los 10 años
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Expansión clonal de célula hematopoyética con:
- Fibrosis medular
- Hematopoyesis extramedular
- Leucoeritroblastosis en Sistema Pulmonar
MIELOFIBROSIS PRIMARIA (MF)
282
ETIOLOGIA: Benceno, radiaciones ionizantes
- Edad 65 años
- Sobrevida 5-6 años
- 1/3 de los ptes asintomáticos
MIELOFIBROSIS PRIMARIA (MF)
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- Intensa **displasia megacariocítica**, **fibrosis** reticulínica en diferentes grados
- Hematopoyesis intrasinusoidal
- Nódulos linfoides
MUTACIÓN: **JAK-2**+ (50%); MPL (5-10%)
MIELOFIBROSIS PRIMARIA (MF)
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MIELOFIBROSIS PRIMARIA (MF)
Clínica:
* Anemia (80%)
* Desconforto abdominal - anorexia
* Sudoración - disminución de peso
* Esplenomegalia severa (90%)
* Hepatomegalia (50-60%)
* Fenómenos autoinmunes: AR,CBP,
* Sjögren
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- Hb < 10g/dl, **dacriocitos** en SP
- Leucoeritroblastosis (75%)
- GB y plaquetas N, aumentadas o
disminuídas
- Aumento LDH, uricemia
MIELOFIBROSIS PRIMARIA (MF)
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MIELOFIBROSIS PRIMARIA (MF)
Causas de muerte:
- Progresión a leucemia (31%)
- Trombosis (14%)
- Infecciones (10%)
- Hemorragias (5%)
- Hipertensión portal (4%)
**Unica opción de curación: trasplante alogénico de MO**
