NORMOCYTIC NORMOCHROMIC ANEMIA Flashcards by Arriane Cyrel (MTI)

APLASTIC ANEMIA
-Otherwise known as

“ Hypoplastic anemia”

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APLASTIC ANEMIA
Characterized by:

-reduction of (?) due to (?) resulting to deficient production of (?)

hematopoietic tissues

hypocellular marrow

blood cells

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APLASTIC ANEMIA
Common signs:

Anemia, infection and bleeding -No splenomegaly and lymphadenopathy

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APLASTIC ANEMIA
Pathogenesis:

A.Direct damage to (?)
B.Immune mediated destruction of (?)
C.Decreased effectiveness/production of (?)

hematopoietic stem cells and progenitor cells

marrow cell

EPO

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APLASTIC ANEMIA Types:

a) Idiopathic Aplastic Anemia

b) Acquired Aplastic Anemia

c) Constitutional Aplastic Anemia

*Familial Aplastic anemia

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with more insidious onset/ unknown origin

a) Idiopathic Aplastic Anemia

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exposure to certain physical and chemical agents, some drugs like antimicrobials, anticonvulsants, analgesics, and radiation (Benezene)

b) Acquired Aplastic Anemia

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**Benzene effect:
(a) metabolism of benzene in the [?];
(b) transport of metabolites to the marrow and their secondary activation to [?] by peroxidase enzymes;
(c) induction of apoptosis, DNA damage and altered differentiation in (?); and
(d) depletion of the [?]

liver

toxic quinones and free radicals

early progenitor cells

stem cell pool

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-associated with congenital anomalies or with genetic predesposition to chronic BM failure

c) Constitutional Aplastic Anemia

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-developmental abnormalities including hyperpigmentation, short stature, hypogonadism, mental retardation, strabismus and malformation

Fanconi’s Anemia (Congenital Aplastic anemia)

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subset of Fanconi’s Anemia.

*Familial Aplastic anemia

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-Pancytopenia, hypocellular marrow but without developmental abnormalities

*Familial Aplastic anemia

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Pure Red Cell Aplasia Subtypes:

*)Transitory Arrest of Erythropoiesis/ Transient aplastic crises
*) Transient Erythroblastopenia of Childhood
*) Acquired Pure red cell aplasia
*) Congenital Red Cell aplasia/Congenital Hypoplastic Anemia (Diamond-Black fan anemia)

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-occur during course of a hemolytiv anemia preceded by an infection with Parvovirus B19

*)Transitory Arrest of Erythropoiesis/ Transient aplastic crises

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-associated with history of viral infections w/n the last 3 months

*) Transient Erythroblastopenia of Childhood

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-associated with thymoma

*) Acquired Pure red cell aplasia

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-caused by a defect in the CFU-E and BFU-E

*) Congenital Red Cell aplasia/Congenital Hypoplastic Anemia (Diamond-Black fan anemia)

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Hematologic classification: Congenital pure red cell aplasia

DIAMOND-BLACKFAN ANEMIA

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Hematologic classification: Congenital aplastic anemia

FANCONI ANEMIA

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Brown skin pigmentation: Uncommon

DIAMOND-BLACKFAN ANEMIA

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Brown skin pigmentation: Common

FANCONI ANEMIA

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Thumb abnormalities: Uncommon

DIAMOND-BLACKFAN ANEMIA

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Thumb abnormalities: Common

FANCONI ANEMIA

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Renal abnormalities: Uncommon

DIAMOND-BLACKFAN ANEMIA

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Renal abnormalities: Common

FANCONI ANEMIA

Onset of hematologic abnormalities: <1 year of age

DIAMOND-BLACKFAN ANEMIA

Onset of hematologic abnormalities: 5-10 years of age

FANCONI ANEMIA

BM biopsy: Cellular

DIAMOND-BLACKFAN ANEMIA

BM biopsy: Hypoplastic to aplastic

FANCONI ANEMIA

BM aspirate: Marked decrease in erythroid precursor only

DIAMOND-BLACKFAN ANEMIA

BM aspirate: Pancytopenia

FANCONI ANEMIA

Peripheral blood: Decrease in RBC,WBC and plt

DIAMOND-BLACKFAN ANEMIA

Peripheral blood: Pancytopenia

FANCONI ANEMIA

Cytogenetics: No assoc abnormalities

DIAMOND-BLACKFAN ANEMIA

Cytogenetics: Multiple chromosomal abnormalities in many tissues

FANCONI ANEMIA

-Normocytic/Normochromic

MYELOPHTISIC ANEMIA

-Associated with marrow replacement by metastatic carcinoma, multiple myeloma or other conditions

MYELOPHTISIC ANEMIA

MYELOPHTISIC ANEMIA -Characterized by the presence of :

normoblast and immature neutropils (leukoerythroblastic anemia)

: inappropriate release of immature erythroid and myeloid due to damage to the underlying marrow stroma by invasion of abnormal cells

**Myelopthisis

-N/N to M/H

ANEMIA OF CHRONIC DISORDER

-Associated with chronic diseases

ANEMIA OF CHRONIC DISORDER

-Decreased EPO production

*Anemia of Renal insufficiency

prominence of burr cells, helmet cell and fragments

*Anemia of Renal insufficiency

Low iron in serum but normal TIBC

*Anemia of Renal insufficiency

-Hypothyroidism

*Anemia of Endocrine diseases

exhbits macrocytic, normochromic cells despite having normal B12 and folate

*Anemia of Endocrine diseases

prominence of acanthocytes due to elevated cholesterol.

*Anemia of Endocrine diseases

-Inherited refractory anemia characterized by eythroid multinuclearity, karyorrhexis and bizarre malformations

Congenital Dyserythropoietic Anemia (CDA)

Congenital Dyserythropoietic Anemia (CDA) Types:

I. Megaloblastic changes w/ some binuclearity II. HEMPAS (Herediatry Eyrhtroblastic Multinuclearity with a Positive Serum Test) III. Giant erythroid precursor w/ pronounced multinuclearity

-most common type

HEMPAS (Herediatry Eyrhtroblastic Multinuclearity with a Positive Serum Test)

-shows binuclearity and multinuclearity

HEMPAS (Herediatry Eyrhtroblastic Multinuclearity with a Positive Serum Test)

-RBC’s possess the “i” blood antigen (HEMPAS antigen)

HEMPAS (Herediatry Eyrhtroblastic Multinuclearity with a Positive Serum Test)

▪ characterized by a severe reduction of the amount of hematopoietic tissues that results to deficient production of blood cells (hypocellular bone marrow)

APLASTIC/HYPOPLASTICANEMIA

▪ rare but potentially fatal bone marrow failure syndrome

APLASTIC/HYPOPLASTICANEMIA

▪ most serious clinical problems relate to neutropenia and thrombocytopenia

APLASTIC/HYPOPLASTICANEMIA

- Anemia with normal mophologies (no problem with iron and hemoglobin)

APLASTIC/HYPOPLASTICANEMIA

- bm fails to produce rbc

APLASTIC/HYPOPLASTICANEMIA

- Otherwise known as " Hypoplastic anemia"

APLASTIC/HYPOPLASTICANEMIA

- Characterized by: ● reduction of hematopoietic tissues ● hypocellular marrow deficient production of red blood cells

APLASTIC/HYPOPLASTICANEMIA

- Common signs: Anemia, infection and bleeding

APLASTIC/HYPOPLASTICANEMIA

- No splenomegaly (no excess production to accomodate extra cells) and no lymphadenopathy

APLASTIC/HYPOPLASTICANEMIA

CHARACTERISTIC FEATURES

▪ no response to EPO (working normal but bm not responding) ▪ reticulocytopenia ▪ bone marrow hypocellularity

- ↓: Hb, Hct, Retic ct

- ↑/N: EPO

PATHOGENESIS

1. Direct damage to the hematopoietic stem and progenitor cells 2. Immune-mediated destruction of marrow cells 3. Decreased effectiveness/production of EPO

Causes: - Chemicals - Accidents (breakage of femur/tissue damage)

- Ab working against the bm damaging the stem cells (less comon)

- No problem in bm but in EPO (more common)

FACTORS AFFECTING IRON ABSORPTION

A1. Idiopathic Aplastic Anemia A2 .Acquired Aplastic Anemia A3. Constitutional Aplastic Anemia

with more insidious (sudden) onset/unknown origin

Idiopathic Aplastic Anemia

has no known cause/origin

Idiopathic Aplastic Anemia

Effects of exposure to: • certain physical and chemical agents: - ionizing radiation - benzene

Acquired Aplastic Anemia

▪ associated with other congenital anomalies (since birth) or with genetic predisposition to chronic bone marrow failure

Constitutional Aplastic Anemia

- occurs in Children

Fanconi's Anemia (Congenital Aplastic Anemia)

features of developmental abnormalities that include: • hyperpigmentation • short stature • hypogonadism • mental retardation • strabismus • malformation of the extremities

Fanconi's Anemia (Congenital Aplastic Anemia)

- occurs in Any age; more severe

Familial Aplastic Anemia

• subset of Fanconi's anemia

Familial Aplastic Anemia

• patients may have pancytopenia and a hypocellular marrow without major developmental abnormalities

Familial Aplastic Anemia

may occur during the course of a hemolytic anemia often preceded by an infection with Parvovirus B19

Transitory Arrest of Erythropoiesis/Transient Aplastic Crises

● Targets mature CFU-E (forming unit of erythrocyte)

Parvovirus B19

● Infects humans (different strain in dogs)

Parvovirus B19

associated with a history of viral infections within the last 3 months having humoral inhibition

Transient Erythroblastopenia of Childhood (TEC)

- Transient suppresion of humoral immune system concerning B lymphocytes ● Ex. Occurs after 3 mos of covid infection ● trasient due to recent viral infection

Transient Erythroblastopenia of Childhood (TEC)

▪ associated with thymoma (tumors of the thymus gland)

Acquired Pure Red Cell Aplasia

▪ Diamond-Blackfan anemia: common name

Congenital Red Cell Aplasia/Congenital Hypoplastic Anemia (Diamond-Blackfan anemia)

▪ caused by a defect in the erythroid-committed progenitor cell (CFU-E and BFU-E) resulting to its accelerated apoptosis

Congenital Red Cell Aplasia/Congenital Hypoplastic Anemia (Diamond-Blackfan anemia)

associated with marrow replacement by metastatic carcinoma, multiple myeloma, or other conditions

anemia that develops in leukemia = normocytic normochromic

MYELOPHTHISIC ANEMIA

- Resembles milary tuberculosis or granuloma

MYELOPHTHISIC ANEMIA

characterized by the presence of varying number of: • normoblasts (↑NRBC) (normoblastic anemia) • immature neutrophils or blast cells of rbc (leukoerythroblastic anemia) • peripheral blood granulocytic (WBC) shift to the left (associated with the appearance of bands, metamyelocytes, and myelocytes; increased blasts/immature cells)

MYELOPHTHISIC ANEMIA

Blood picture: • usually normocytic-normochromic • occasionally microcytic-hypochromic

ANEMIA OF CHRONIC DISORDERS

ASSOCIATED CHRONIC DISEASES

*Anemia of Renal insufficiency *Anemia of Liver diseases *Anemia of Endocrine diseases

- N/N to M/H

- Inc cytokine - Dec EPO - Altered iron metabolism (microcytic)

▪ megaloblastic changes with some binuclearity (N-C asynchrony)

CDA TYPE I

▪ mild to severe chronic anemia

CDA TYPE I

▪ caused by mutations in the CDAN1 or C15orf41 genes

CDA TYPE I

- chromosome 15

CDA TYPE I CDA TYPE III

▪ Hereditary Erythroblastic Multinuclearity with a Positive Acid Serum Test (HEMPAS)

CDA TYPE II

• most common subtype

CDA TYPE II

• red cells show binuclearity and multinuclearity

CDA TYPE II

▪ RBCs possess the i-blood antigen (HEMPAS antigen) --- severe hemolysis

CDA TYPE II

▪ results from mutations in the SEC23B gene

CDA TYPE II

- chromosome 20 (most common type of CDA)

CDA TYPE II

▪ least common of the CDA subtypes

CDA TYPE III

▪ giant erythroid precursor with more pronounced multinuclearity (blast cell divides, but w/o daughter cell; nulceus divides similar to megakayocyte appearance)

CDA TYPE III

associated with mutations in the KIF23 gene, which codes for a protein involved in cytokinesis

CDA TYPE III

NORMOCYTIC NORMOCHROMIC ANEMIA Flashcards

(106 cards)