QUANTITATIVE RED CELL DISORDERS PART II Flashcards
- are macrocytic anemias in which DNA is unimpaired
- lack hypersegmented neutrophils and oval macrocytes in the peripheral blood and megaloblasts in the bone marrow
Macrocytic nonmegaloblastic anemias
Normocytic normochromic anemia
Bone marrow failure
cells have increased expression of Fas receptors that mediate apoptosis and increased expression of apoptosis- related genes
CD34+ aplastic anemia
-elevated blood and bone marrow cytotoxic T cells with an oligoclonal expansion of specific T-cell clones
- increased T cell production of such cytokines as interferon-Ξ³ and tumor necrosis factor-Ξ±
- upregulation of T-bet
- increased TNF-Ξ± receptors on CD34+ cells
- improvement in cytopenias after immunosuppressive therapy
Autoimmune pathophysiology: Acquired Aplastic Anemia
Symptoms:
- insidious-onset anemia with pallor, fatigue, and weakness
- tachycardia, hypotension, cardiac failure, death
- petechiae, bruising, epistaxis, mucosal bleeding, menorrhagia, retinal hemorrhages, intestinal bleeding, intracranial hemorrhage
- fever and bacterial/fungal infections
APLASTIC ANEMIA
decreased neutrophils, monocytes, and platelets
APLASTIC ANEMIA
RBCs are macrocytic or normocytic
APLASTIC ANEMIA
- toxic granulation in neutrophils may be observed
- serum iron level and percent transferrin saturation are increased
- erythroid, granulocytic, and megakaryocytic cells are decreased/ab
APLASTIC ANEMIA
present at an earlier age and may have characteristic physical stigmata
Inherited Aplastic Anemia
- is a chromosome instability disorder characterized by aplastic anemia, physical abnormalities, and cancer susceptibility
Fanconi Anemia
skeletal abnormalities (thumb malformations, radila hypoplasia, microcephaly, hip dislocation, scoliosis)
Fanconi Anemia
- skin pigmentation (hyper-/hypopigmentation, cafe-au-lait lesions)
- short stature, abnormalities of the eyes, kidneys, and genitals
- low birth weight and developmental delay
Fanconi Anemia
-characterized by mucocutaneous abnormalities, bone marrow failure, and pancytopenia
- triad of abnormal skin pigmentation, dystrophic nails, and oral leukoplakia
Dyskeratosis Congenita
Multisystem abnormalities: pulmonary fibrosis, liver disease, developmental delay, short stature, microcephaly, prematurely gray hair or hair loss, immunodeficiency dental carries, periodontal disease
Dyskeratosis Congenita
an inherited multisystem disorder characterized by pancreatic insufficiency, cytopenia, skeletal abnormalities, and a predisposition for hematologic malignancies
Shwachman-Bodian-Diamond Syndrome
3 types of Inherited Aplastic Anemia
- Fanconi Anemia
- Dyskeratosis Congenita
- Shwachman-Bodian-Diamond Syndrome
- a rare disorder of erythropoiesis characterized by a selective decrease in erythrocyte precursors in an otherwise normal bone marrow
PURE RED CELL APLASIA
Acquired Pure Red Cell Aplasia:
Transient Erythroblastopenia of Childhood
Take note
the acquired form of PRCA in young children
Transient Erythroblastopenia of Childhood
Diamond-Blackfan Anemia
Congenital Pure Red Cell Aplasia:
- a congenital erythroid hypoplastic disorder of early infancy
- approximately half of patients have: physical anomalies (craniofacial dysmorphisms, short stature, neck and thumb malformations)
Diamond-Blackfan Anemia
- are a heterogeneous group of rare disorders characterized by refractory anemia, reticulocytopenia, hypercellular bone marrow with markedly ineffective erythropoiesis, distinctive dysplastic changes in bone marrow erythroblasts
CONGENITAL DYSERYTHROPOIETIC ANEMIA
is due to the infiltration of abnormal cells into the bone marrow and subsequent destruction and replacement of
normal hematopoietic cells
MYELOPHTHISIC ANEMIA
- common complication of CKD is anemia
- burr cells are common peripheral blood film findings
1ANEMIA OF CHRONIC KIDNEY DISEASE
