1. Thalassemia is a heterogeneous group of inherited disorders characterized by decreased synthesis of the alpha or beta globin chains of hemoglobin A, leading to ineffective erythropoiesis and hemolysis.
2. Beta-thalassemias result from reduced or absent beta chain synthesis and include beta-thalassemia major (severe transfusion dependent anemia), beta-thalassemia intermedia (severe anemia but not transfusion dependent), and beta-thalassemia minor (asymptomatic with mild anemia).
3. Laboratory findings in beta-thalassemia major include severe anemia, peripheral blood changes, increased HbF and HbA2 on electroph
In this presentation I've tried to summarize classification of hemolytic anemia and in depth review of rbc membrane disorders like hereditary spherocytosis, hereditary elliptocytosis, enzymopathies of hemolytic anemia like g6pd disorder, pyruvate kinase disorders, hemoglobinopathies related to hemolytic anemia like thalassemia, sickle cell anemia and especially pathophysiology and mechanism of hemolysis either extravascular or intravascular. Hope it helps you understand the entity better.
In this presentation I've tried to summarize classification of hemolytic anemia and in depth review of rbc membrane disorders like hereditary spherocytosis, hereditary elliptocytosis, enzymopathies of hemolytic anemia like g6pd disorder, pyruvate kinase disorders, hemoglobinopathies related to hemolytic anemia like thalassemia, sickle cell anemia and especially pathophysiology and mechanism of hemolysis either extravascular or intravascular. Hope it helps you understand the entity better.
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The Roman Empire, a vast and enduring power, stands as one of history's most remarkable civilizations, leaving an indelible imprint on the world. It emerged from the Roman Republic, transitioning into an imperial powerhouse under the leadership of Augustus Caesar in 27 BCE. This transformation marked the beginning of an era defined by unprecedented territorial expansion, architectural marvels, and profound cultural influence.
The empire's roots lie in the city of Rome, founded, according to legend, by Romulus in 753 BCE. Over centuries, Rome evolved from a small settlement to a formidable republic, characterized by a complex political system with elected officials and checks on power. However, internal strife, class conflicts, and military ambitions paved the way for the end of the Republic. Julius Caesar’s dictatorship and subsequent assassination in 44 BCE created a power vacuum, leading to a civil war. Octavian, later Augustus, emerged victorious, heralding the Roman Empire’s birth.
Under Augustus, the empire experienced the Pax Romana, a 200-year period of relative peace and stability. Augustus reformed the military, established efficient administrative systems, and initiated grand construction projects. The empire's borders expanded, encompassing territories from Britain to Egypt and from Spain to the Euphrates. Roman legions, renowned for their discipline and engineering prowess, secured and maintained these vast territories, building roads, fortifications, and cities that facilitated control and integration.
The Roman Empire’s society was hierarchical, with a rigid class system. At the top were the patricians, wealthy elites who held significant political power. Below them were the plebeians, free citizens with limited political influence, and the vast numbers of slaves who formed the backbone of the economy. The family unit was central, governed by the paterfamilias, the male head who held absolute authority.
Culturally, the Romans were eclectic, absorbing and adapting elements from the civilizations they encountered, particularly the Greeks. Roman art, literature, and philosophy reflected this synthesis, creating a rich cultural tapestry. Latin, the Roman language, became the lingua franca of the Western world, influencing numerous modern languages.
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The US House of Representatives is deeply concerned by ongoing and pervasive acts of antisemitic
harassment and intimidation at the Massachusetts Institute of Technology (MIT). Failing to act decisively to ensure a safe learning environment for all students would be a grave dereliction of your responsibilities as President of MIT and Chair of the MIT Corporation.
This Congress will not stand idly by and allow an environment hostile to Jewish students to persist. The House believes that your institution is in violation of Title VI of the Civil Rights Act, and the inability or
unwillingness to rectify this violation through action requires accountability.
Postsecondary education is a unique opportunity for students to learn and have their ideas and beliefs challenged. However, universities receiving hundreds of millions of federal funds annually have denied
students that opportunity and have been hijacked to become venues for the promotion of terrorism, antisemitic harassment and intimidation, unlawful encampments, and in some cases, assaults and riots.
The House of Representatives will not countenance the use of federal funds to indoctrinate students into hateful, antisemitic, anti-American supporters of terrorism. Investigations into campus antisemitism by the Committee on Education and the Workforce and the Committee on Ways and Means have been expanded into a Congress-wide probe across all relevant jurisdictions to address this national crisis. The undersigned Committees will conduct oversight into the use of federal funds at MIT and its learning environment under authorities granted to each Committee.
• The Committee on Education and the Workforce has been investigating your institution since December 7, 2023. The Committee has broad jurisdiction over postsecondary education, including its compliance with Title VI of the Civil Rights Act, campus safety concerns over disruptions to the learning environment, and the awarding of federal student aid under the Higher Education Act.
• The Committee on Oversight and Accountability is investigating the sources of funding and other support flowing to groups espousing pro-Hamas propaganda and engaged in antisemitic harassment and intimidation of students. The Committee on Oversight and Accountability is the principal oversight committee of the US House of Representatives and has broad authority to investigate “any matter” at “any time” under House Rule X.
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2. Adult hemoglobin - 96% HbA (2 2)
• Adult Hb is tetramer of 2 -chains & 2
-chains encoded by;
- Pair of -globin genes on chr. 16
- Single -globin gene on chr.11
2
2
2
2
Hemoglobin Synthesis
3. •Heterogeneous group of inherited disorders
characterized by decreased synthesis of
either or -globin chain of HbA (2 2) leading
to
- Low Intracellular hemoglobin
(Hypochromia)
- Relative excessive of unimpaired globin
chains Ineffective erythropoiesis & Hemolysis
Thalassemias
4. • Most Frequent in Mediterranean,
African, or Asian Populations
• -Thalassemias : Chain Synthesis
(100 different causative mutations)
• -Thalassemias : Chain Synthesis
Deletions are more common (SE Asians)
Thalassemias
5. • Diminished synthesis of structurally
normal -globin chains, coupled with
unimpaired synthesis of chains which
aggregate into insoluble inclusions within
RBC & their precursors
Ineffective erythropoiesis & Hemolysis
-Thalassemias
6.
7.
8. 1. Thalassemia Major : Severe transfusion
dependent anemia
i) Homozygous 0 – Thalassemia (0/0)
ii) Homozygous + - Thalassemia (+/+)
2. Thalasemia intermedia : Severe anemia but not
transfusion dependent
- Heterozygous with one - Thalassemia gene &
one normal gene : (+/ or 0 /)
3. Thalassemia minor : (- Thalassemia trait):
Asymptomatic with mild or absent anemia
- Heterozygotes, milder variants (+/ or 0 /)
-Thalassemias-Syndromes
9.
o o
+ +
No Chain
Synthesis
Severe
Chain
Synthesis
Severe Anemia
Chromosome 11
- Thalassemia Major
10. i) Promoter region mutations :
Transcription defect:
- + Thalassemias (Some normal -globin
is synthesized)
ii) Chain terminator mutations :
Translation defect: Premature termination of mRNA
translation
- 0 Thalassemia (Prevention of -globin
synthesis)
iii) Splicing mutations : Most Common:
Both + & 0 Thalassemias occur
-Thalassemias -Molecular Pathogenesis
11. • Anemia manifests 6 to 9 months after
birth, as Hb synthesis switches from HbF to
HbA
• Marked erythroid hyperplasia
Expansion of bones Facial bone
deformity ( Mongoloid facies/Thalasemia
facies ) & malocclusion of jaw
• Extramedullary hematopoiesis involving
liver, spleen & lymphnodes massive
enlargement
• Iron overload (Repeated blood transfusions
& absorption of dietary iron)
Hemosiderosis involving mainly heart, liver
& pancreas
-Thalassemia-Major Clinical Features
12.
13.
14.
15.
16.
17.
18.
19. 1. Anemia usually severe (Hb 3 to 6
gm/dl)
2. Peripheral blood :
Marked anisocytosis with
predominant microcytes, severe
hypochromia, basophilic
stippling, plenty of target cells &
nucleated RBC
3. Reticulocytosis
4. MCV, MCH & MCHC
-Thalassemia Major-Laboratory Findings
20. 5. Serum bilirubin (Unconjugated)
6. Osmotic fragility – Decreased
( resistant to saline hemolysis)
7. Bone marrow : Erythroid(Normoblastic)
hyperplasia / Increased iron stores
8.Hb electrophoresis :
-Increased HbF & HbA2
-complete absence or small amounts of
HbA
-Thalassemia Major- Laboratory Findings
21.
o
+
chain
synthesis
chain
synthesis
Mild or no anemia
- Thalassemia Minor
(Asymptomatic -Thalassemia trait)
22. Laboratory Findings :
• Mild anemia
• Peripheral blood :Microcytosis & hypochromia,
occasional target cells
• Mentzer index:MCV/RBC >13 indicates IDA ; < 13
in Thal.minor
• MCV, MCH & MCHC – Reduced
• Hb electrophoresis : Confirmatory; Increase in
HbA2 to 4 to 8% of total Hb (Normal 2.5
%); HbF- N or slightly
- Thalassemia Minor
(Asymptomatic -Thalassemia trait)
23.
24. 1. Differentiation from hypochromic,
microcytic anemia of Iron deficiency
(serum Iron, TIBC & Ferritin estimation)
2. Genetic counseling
For individuals (Women of child bearing
age) at risk for both Thalassemia trait &
Iron deficiency Hb electrophoresis gives
confirmatory diagnosis
- Thalassemia Minor
Importance of Recognition of -Thalassemia trait:
26. • Hb Barts: -Globin chain tetramer (4)
: High oxygen affinity severe tissue
hypoxia
• Fatal in utero or infant dies shortly
after birth
• Severe anemia with microcytic,
hypochromic picture
• Hb electrophoresis : 80-90 % Hb-Barts
& small amounts of Hb-H & Hb-
Portland; No HbA, HbA2 or HbF
Hb Barts-Hydrops Fetalis
