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Pathology > Pathology of the hematopoietic and lymphoid systems > Flashcards

Pathology of the hematopoietic and lymphoid systems Flashcards

1
Q

Causes of IDA

A
  • Chronic blood loss (from i.e. the GI tract (ulcers, cancer, hemorrhoids) or the female genital tract (menorrhagia, cancers))
  • Low intake
  • Increased demand (pregnancy, childhood growth etc.)
  • Malabsorption syndrome (pancreatic insufficiency, celiac disease etc.)
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2
Q

Diagnosis of IDA

A
  • Hypochromic and microcytic RBCs
  • Low serum ferritin and iron
  • Low transferrin saturation
  • Increased total iron-binding capacity
  • Response to iron therapy
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3
Q

Morphology of megaloblastic anemia

A
  • Anemia (
  • Increased MCV, due to inability to create DNA (macrocytic anemia - cells grow, without dividing)
  • Hypersegmented neutrophils
  • Elevated homocysteine level
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4
Q

Pathogenesis of pernicious anemia (VB12 deficiency)

A

Autoimmune disease that destroys parietal cells, leading to less intrinsic factor being produced. Less IF leads to less B12 absorption, thus deficiency.

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5
Q

Diagnosis of pernicious anemia

A
  • Low serum VB12
  • Normal/elevated serum folate levles
  • Serum antibodies to IF
  • Moderate to sevre megaloblastic anemia
  • Leukopenia with hypersegmented granulocytes
  • Dramatic reticulocyte response to VB12 administration
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6
Q

Difference between folate and VB12 deficiency

A

VB12 deficiency has all the characteristics of folate deficiency, and additional neurologic symptoms (demyelinating disorder to be be precise)

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7
Q

Pathogenesis of aplastic anemia

A
  • Usually idiopathic (i.e. viral infection)

- Other cases are related to exposure to a myelotoxic agent

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8
Q

Morphology of aplastic anemia

A
  • Hypocellular bone marrow (only lymphocytes and plasma cells seen)
  • Can cause fatty change in the liver
  • Thrombocytopenia can cause hemorrhages
  • Granulocytopenia can cause bacterial infections
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9
Q

Anemia of chronic disease: possible causes

A
  • Chronic microbial infections
  • Chronic immune disorders
  • Neoplasms
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10
Q

Anemia of chronic disease: pathogenesis

A

High levels of plasma hepcidin (due to pro-inflammatory cytokines) blocks the transfer of iron to erythroid precursors. Additionally, chronic inf. blocks erythropoietin synthesis in the kidneys.

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11
Q

Anemia of chronic disease: clinical manifestations

A
  • Low serum iron levels

- RBCs: hypochromic (pale) and microcytic (small)

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12
Q

Myelophtisic anemia: cause of morphology

A

Caused by intensive infiltration of the bone marrow by tumors.

Morphology:

  • Misshaped RBCs
  • Thrombocytopenia
  • Leukoerythroblastosis (immature granulocytic and erythrocytic precursors with mild leukocytosis)
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13
Q

Heridetary spherocytosis: pathogenesis

A

And AD abnormality in the membrane skeleton of erythrocytes, featured by mutations in the proteins spectrin (responsible for maintenance of the RBC shape) and ankyrin)

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14
Q

Heridetary spherocytosis: morphology

A
  • Spherocytes are dark red without central pallor
  • Compensatory hyperplasia of RBCs due to excessive destruction
  • Splenomegaly (due to congestion of splenic cords)
  • If long-standing: hemosiderosis
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15
Q

Heridetary spherocytosis: clinical features

A
  • Anemia
  • Splenomegaly
  • Jaundice
  • Spherocytes show increased osmotic fragility in mild hypotonic salt solutions
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16
Q

Sickle cell anemia

A

Low oxygen saturation promotes RBC sickling. Repeated episodes of sickling damages the cell membrane and decreases elasticity, thus the RBCs fail to restore to the normal shape when oxygen saturation is back to normal.

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17
Q

Consequences of sickling

A
  • Chronic hemolytic anemia: caused by RBC membrane damage and dehydration
  • Widespread microvascular obstructions, which further cause ischemia and pain crises
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18
Q

Sickle cell anemia: organ changes

A
  • Fatty changes due to decreased oxygen: in the heart, liver and renal tubules
  • Compensatory hyperplasia of erythroid progenitors. Can further cause bone resorption and secondary bone formation.
  • Extramedullary hematopoiesis
  • Splenomegaly
  • Hemosiderosis and gallstones
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19
Q

Types of vaso-occlusive (pain) crisis seen in sickle cell anemia

A
  • In the bone marrow, where it can cause infarcation
  • Acute chest syndrome: sickling in pulmonary beds –> hypoxia
  • Stroke: in generally any organ damaged by ischemia
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20
Q

β-thalassemia minor and α-thalassemia: genetics, morphology and clinical features

A

β-thalassemia minor: inheritance of one abnormal allele
α-thalassemia: loss of one or more gene (severity depends on number of missing genes)

Abnormalities are confined to the peripheral blood, where RBCs appear microcytic and hypochromic with a regular shape. Usually asymptomatic.

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21
Q

β-thalassemia intermedia/HbH disease: genetics, morphology and clinical features

A

Inheritance of two β+ alleles

Smears between the two extremes (see other cards). Anemia is moderate, without the need for transfusions

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22
Q

β-thalassemia major: genetics, morphology and clinical features

A

Inheritance of two β0 and β+ alleles

Smear: microcytosis, hypochromia, piokilocytosis (variation in cell size) and anisocytosis.

Clinically:

  • Growth retardation in children
  • Systemic iron overload can cause cardiac dysfunction due to secondary hemochromatosis, which further causes death.
  • Treated best by an early BM transplant
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23
Q

Anatomical changes cause by thalassemia

A
  • Skeletal deformities, due to expanded erythropoietic marrow
  • Hepatosplenomegaly, due to extramedullar hematopoiesis
  • Lymphadenopathy
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24
Q

G6PDH deficiency: pathogenesis

A

Triggered by exposure to infectious agents or drugs. GSH (glutathione), which normally inactivates pathogens, cannot do so, thus oxidants continue to damage RBC components, especially hemoglobin. Oxidized hemoglobin forms Heinz bodies, which further damage the cell membrane and causes intravascular hemolysis.

Extravascular hemolysis results when splenic macrophages try to remove the Heinz bodies, forming bite cells which are trapped in the spleen.

25
Q

Genetics of G6PDH deficiency

A

X-linked, thus more common in males. A mutation in G6PD A- is most common.

26
Q

Warm antibody immunhemolytic anemia

A
  • Caused by IgG (active at 37°C)
  • Usually idiopathic
  • Opsonization of RBCs by autoantibodies causes erythrophagocytosis in the spleen. Cell membrane loss results in formation of spherocytes, which are destroyed in the spleen.
27
Q

Cold antibody immunhemolytic anemia

A
  • Caused by IgM (active below 30°C)
  • RBCs travel to warmer body areas, where IgM is released, causing opsonization. Mostly in the spleen and liver (extravascular hemolysis).
28
Q

Leukopenia: pathogenesis

A

Decreased granulocyte production:

  • BM failure
  • Tumor growth into the BM
  • Chemotherapy

Increased granulocyte destruction:

  • Immune-mediated injury
  • Infection
  • Splenomegaly
29
Q

Leukopenia: morphology

A

Marrow hypercellularity with excessive neutrophil destruction or ineffective granulopoiesis

30
Q

Leukopenia: clinical features

A

First: malaise, chills and fever. Then: weakness and fatigue. Infections which are ulcerating and necrotizing can be seen in the oral cavity or pharynx.

31
Q

Causes of neutrophilic leukocytosis

A
  • Acute bacterial infections

- Sterile inflammation (tissue necrosis, burns)

32
Q

Causes of eosinophilic leukocytosis

A
  • Allergies
  • Allergic skin diseases
  • Parasitic infections
  • Drug reactions
  • Malignancies
33
Q

Causes of monocytosis

A
  • Chronic infections (i.e. TBC)
  • Bacterial endocarditis
  • Rickettsiosis, malaria
  • IBD
34
Q

Causes of lymphocytosis

A

Usually accompanies monocytosis in disorders associated with chronic immunologic stimulation.

  • Viral infections
  • Bordetella pertussis infection
35
Q

Infective mononucleosis: pathogenesis

A

Infection due to the EBV virus

Infects the oropharynx, then spreads to the underlying lymph nodes to infect mature B cells. B cells undergo polyclonal activation and proliferation to secrete different antibodies.

A characteristic finding is virus-specific CD8+ T cells (atypical lymphocytes)

36
Q

Infective mononucleosis: morphology

A
  • Peripheral blood leukocytosis: more than half are atypical lymphocytes
  • Lymphadenopathy: filled with atypical lymphocytes
  • Spleen: enlarged and infiltrated with lymphocytes
  • Liver: atypical lymphocyte infiltration
37
Q

Features of acute nonspecific lymphadenitis

A
  • Can be isolated or generalized
  • Inflamed nodes are swollen, grey-red and have large GCs
  • In case of cause being a pyogenic organism, there is an infiltration of neutrophils around the GCs
  • If severe, follicular centers can necrotize
38
Q

Patterns of chronic nonspecific lymphadenitis (3)

A
  1. Follicular hyperplasia: infection activates B cells, causing a follicular/GC reaction. LN architecture remains normal. GC lymphocytes are of varied shape and size.
  2. Paracortical hyperplasia: immune reation in the T cell regions. GC reduces in size.
  3. Sinus histiocytosis: distention of lymphatic sinusoids, due to hypertrophy of lining endothelial cells and infiltrating macrophages
39
Q

Cat-scratch disease (cause, symptoms, complications, morphology)

A

Caused by the bacteria Bartonella henselae

Main symptom: regional lymphadenopathy

Complications: encephalitis, osteomyelitis or thrombocytopenia

Morphology:

  • Nodal changes: first a sarcoid-like granuloma, causing central necrosis due to neutrophil infiltration, causing stellate necrotizing granulomas, eventually leading to abscess formation
  • The microbe can be visualized with a silver stain
40
Q

Toxoplasmosis (cause, transmission, symtoms, diagnosis)

A

Caused by the parasite Toxoplasma gondii.

Oral-fecal transmission, i.e. from eating infected meat, water or soil infected with animal feces.

Asymptomatic or flu-like symptoms.

Can be difficult to differentiate from a granuloma, thus PCR should be performed to detect the parasite.

41
Q

CML: mutation

A

t(9,22) creates a BCR-ABL fusion gene (ABL moves to the BCR part on chromosome 22)

42
Q

CML: morphology

A
  • Elevated leukocyte count
  • Hypercellular bone marrow (increased granulocytic and megakaryocytic precursors)
  • Enlarged spleen
43
Q

CML: clinical features

A
  • Nonspecific symptoms

- Differentiate from a leukemoid reaction using karyotyping, FISH or PCR

44
Q

Phases/progression of CML

A

Begins with a chronic phase. 50% progress to an accelerated phase with increased anemia, new thrombocytopenia, more cytogenetic abnormalities. Then there’s a blast crisis: 30% look like B-ALL, 70% look like AML. Rarely progresses to a phase of extensive BM fibrosis.

45
Q

CML: therapy

A

Chemotherapy decreases the WBC count. Gene therapy (imatinib) will suppress the neoplastic clone, although the mutation remains.
BCR-ABL tyrosine kinase inhibitors can also be used.

46
Q

Primary Myeloid Fibrosis: pathogenesis

A

Fibroblast proliferation is stimulated by platelet derived growth factor and transforming growth factor β.

JAK2 mutation found in 50% of cases.

47
Q

Primary Myeloid Fibrosis: morphology

A

On smear:

  • Poikilocytes (strangely shaped RBCs)
  • Leukoerythroblastosis
  • Abnormally large platelets
  • Abnormally shaped megakaryocytes seen in areas of extramedullary hematopoiesis
48
Q

Primary Myeloid Fibrosis: organ changes

A

Extramedullary hematopoiesis is related to enlargemenet of the spleen, liver and to some extent lymph nodes.

The bone marrow is initially hypercellular with focal areas of fibrosis. When advanced, it becomes hypocellular and diffusely fibrotic.

49
Q

Essential Thrombocytopenia

A
  • Thrombocytosis: increased platelet count
  • Usually due to a JAK2 mutation
  • BM: atypical, huge megakaryocytes
  • No therapy is needed, but there’s an increased chance for thrombosis
  • Treatment: plasmaphoresis
50
Q

Polycythemia Vera: pathogenesis

A

Associated with low levels of erythropoietin and activating mutations in JAK2.

51
Q

Polycythemia Vera: morphology

A

Increased blood volume and viscosity. Further leads to:

  • Enlarged spleen and liver (extramedullary hematopoiesis)
  • Thromboses and infarctions are common
  • Hemorrhages (in the GIT, oropharynx or brain)
  • Smear shows basophilia (similar to CML)
  • Hypercellular BM
  • Trousseau’s phenomenon (thrombosis in veins and sinusoids)
52
Q

Polycythemia Vera: clinical course

A

Patients are plethoric (congested, ruddy) and cyanotic.

Complaints related to thromboses and hemorrhages: HTN, headache, dizziness etc.

53
Q

Polycythemia Vera: diagnosis

A
  • RBC count: 6-10 million /microliter
  • Hematocrit increased by 60%
  • Basophilia
  • Thrombotic complications
  • Budd-Chiari syndrome (from hepatic vein thrombosis; rare)
  • Can evolve into a “spent phase”, whereas it resembles primary myelofibrosis
54
Q

Definition of massive splenomegaly and examples of disorders causing it

A

Weight: > 1000g

  • Myeloproliferative disorders (CML, PMF)
  • CLL and HCL
  • Lymphomas
55
Q

Definition of moderate splenomegaly and examples of disorders causing it

A

Weight: 500-1000g

  • Chronic congestive splenomegaly
  • Acute leukemias
  • Heridetary spherocytosis
  • TBC, sarcoidosis, typhoid fever
56
Q

Definition of mild splenomegaly and examples of disorders causing it

A

Weight: < 500g

  • Acute splenitis
  • Congestion
  • Infectious mononucleosis
57
Q

Causes and definition of hypersplenism

A

Definition: increased removal of cellular blood components.

Causes:

  1. Disorders causing widening of splenic cords:
    - Congestive splenomegaly
    - Gaucher’s
    - Leukemias and lymphomas
  2. Infections
  3. Abnormal cellular blood components
    - Heridetary spherocytosis
58
Q

Significance of thymic hyperplasia

A

Due to lymphoid follicles or germinal centers becoming present in the medulla.

Oten seen in people with myasthenia gravis or, rarely, other autoimmune disorders.

59
Q

Distinguish between type I and II thyomas

A

Type I: cytologically benign, showing infiltration and local aggression. Can metastasize. Composed of epithelial cells and reactive thymocytes.

Type II (thymic cc): fleshy, invasive masses. Often metastasizes to the lungs. Resembles squamous cell cc.

Pathology (14 decks)

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