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๐Ÿ”ด๐Ÿ”ดSpherical cells in Blood Film ?

Not only Spherocytosis .

It maybe AIHA

Or Macrocytic spherocytes in non Megaloblastic macrocytosis

#ุจุงุทู†ุฉ
Schizocyte = Helmet cells = fregmented cells

ุฎู„ุงูŠุง ู…ุชูƒุณุฑุฉ .. ูƒุงู†ุช ุทุจูŠุนูŠุฉ ูˆ ุนุงูŠุดุฉ ุญูŠุงุชู‡ุง ู„ู‚ุช ู‚ุฏุงู…ู‡ุง ู‚ุทุงุนูŠู† ุทุฑู‚ ูƒุณุฑูˆู‡ุง ๐Ÿ˜”๐Ÿ˜”

Causes:-

MAHA ๐Ÿ˜โค๏ธ

๐Ÿ”บM i cro - micro Angiopathic hemolytic anemia :-

May be :-

- DIC
- TTP
-HUS
- HELLP syndrome in pregnancy

Or

๐Ÿ”บM Acro - macro angiopathic Heamolytic anemia.

Prosthetic like : metalic valve

#ุจุงุทู†ุฉ
Howell- jolly Body :-

Howell-Jolly bodies are nuclear remnants that are found in the RBCs of patients with reduced or absent splenic function (e.g., sickle cell disease, heterotaxy) and in patients with megaloblastic anemias. They are occasionally present in the RBCs of premature infants.

ุงูŠุด ููƒุฑุชู‡ุงุŸ!
ู‡ูŠ ุนุจุงุฑุฉ ุนู† ุจู‚ุงูŠุง ููŠ ุงู„ DNA ... ู…ู…ูƒู† ู†ุญุตู„ู‡ุง ููŠ ุญุงู„ ูƒุงู† ุงู„ DNA ู†ูุณู‡ ู…ุด ู‚ุงุฏุฑ ูŠู†ู‚ุณู… ุณูˆุง ู…ุซู„ ุญุงู„ุงุช ุงู„ Megaloblastic anemia ูˆู†ู‚ุต vitamin B12 , folic acids ..
ุทูŠุจ ููŠ ุงู„ูˆุถุน ุงู„ุทุจูŠุนูŠ ..
ูŠุดูˆูู‡ู… ุงู„ Spleen ูˆูŠุชุฎู„ุต ู…ู†ู‡ู… ..
ุงุฐุง ู…ู…ูƒู† ู†ุดูˆูู‡ู… ุจุฑุถูˆ ููŠ ุญุงู„ุงุช Post Splenectomy ุงูˆ SCA

1โƒฃMegaloblastic anemia
2โƒฃSCA
3โƒฃPost splenectomy
4โƒฃ Splenic Atropy like in : Celiac disease
5โƒฃpremature neonate

#ุจุงุทู†ุฉ
#ุฃุทูุงู„
๐Ÿ›‘Blood film of Megaloblastic anemia:-

1- RBCs :- Macro-ovalocyte

2- WBCs :- Hypersegmented Neutrophils

3- Howell- jolly bodies

4- Tear drop cells

#ุฃุทูุงู„
#ุจุงุทู†ุฉ
โšช๏ธ Sidroblastic anemia:-

( Defect in protoporphyrin synthesis)

We need Protoporphyrin + Irom to form= Haemoglobin

ู‡ู†ุง ู…ุง ุนุงุฏ ุนูŠุชูƒูˆู†ุด ุงู„ Protoporphyrin !

ูˆุงู„ุณุจุจุŸ
ุงู„ุงู†ุฒูŠู… ุงู„ู„ูŠ ุงุณู…ู‡
" Aminolevulinic acid synthase "

ู…ุง ูŠุดุชุบู„ ุณูˆุง ูˆู‡ูˆ ูŠุญุชุงุฌ ุงู„ู‰ Vitamin B6 ( (pyridoxine)

ุงู„ู„ูŠ ุจูŠุตูŠุฑ ุงู†ูˆ ูŠุธู„ ุงู„ Iron ู…ุชุฑุงูƒู… ุจุงู„ Cells ูˆูŠูƒูˆู† Sidroblastic ring ููŠ ุงู„ BM

๐Ÿ”น Causes ?

1- Hereditary [ X- linked ]
2- Deficiency of Vitamin B6
3- Lead Poisoning
4- Alcoholism
5- Drugs like Anti- TB : Isoniazid.

๐Ÿ”น Findings:-

- Anemia menifestations

- High serum iron , High serum ferritin , high transferrin , low TIBC

- the presence of bone marrow ring sideroblasts, erythroblasts with coarse blue granules at Perls staining " Pappenheimer bodies "

๐Ÿ”น treatment:-
- Vitamin B6
- Blood transfusion

#ุฃุทูุงู„
#ุจุงุทู†ุฉ
๐Ÿ‘€In Megaloblastic anemia:-

- to know weither it is Vitamin B12 or B9 ..

โ†—๏ธ Homocysteine in Both deficiencies

โ†—๏ธ ( MMA ) Methylmalonic acid ( in Vitamin B12 ) deficiency

๐Ÿ”ต Note :-

When you treat the patient you must give him both Vitamin B9 + B12

ู„ุงู† B9 ูŠุนุชู…ุฏ ููŠ ุนู…ู„ู‡ ุนู„ู‰ ุงู„ B12 .
ูˆุนุดุงู† ูŠุชุญูˆู„
Dihydrofolate >> tetrahydrofloate
ูŠุญุชุงุฌ ุฅู„ู‰ B12

ูˆู„ูˆ ุนุงู„ุฌู†ุง ุจ ููŠุชุงู…ูŠู† B9 ูู‚ุท ูˆ ุนู†ุฏู†ุง ู†ู‚ุต ููŠ B12 ู†ุฏุฎู„ ุงู„ู…ุฑูŠุถ ููŠ Folate Trap .

#ุจุงุทู†ุฉ
๐ŸŸกHereditary Spherocytosis:-

Autosomal Dominant .

Defect in mambrane proteins ( Spectrins , Ankyrins) >> abnormal shape >> engulfed by spleen [ Extravascular Hemolysis]

ููŠ ู‡ุฐูŠ ุงู„ุญุงู„ุฉ ูŠูƒูˆู† :-
Splenectomy corrects the anemia ..

ูˆููŠ ู‡ุฐูŠ ุงู„ู…ุฑุถ ุชูƒูˆู† ุฎู„ุงูŠุง ุงู„ุฏู… Fragile ..
ุชุชูƒุณุฑ ุจุณู‡ูˆู„ุฉ ู„ุงู†ูˆ ุงู„ Mambrane ู†ุงู‚ุต ุงู„ุจุฑูˆุชูŠู†ุงุช
ู„ู‡ุฐุง ู†ุฃูƒุฏ ุงู„ู…ุฑุถ ุจุงู„ูุญุต :-
Osmotic fragility test

ูˆุชูƒูˆู† ุงู„ุฎู„ุงูŠุง ุจุงู„ smear:-
Spherocytes [ Hyperchromia ]

#ุจุงุทู†ุฉ
G6PD is intravascular heamolysis

Due to lack of protection aganist the oxidative stress aganist RBCs

X-linked Recessive .

On blood Film :-

ุงู„ Oxidative stress ุจูŠู‡ุงุฌู… ุฎู„ุงูŠุง ุงู„ุฏู… ... ุจุชูƒูˆู† ุฒูŠ ุงู„ู…ุฃูƒูˆู„ุฉ Bite cells ุงูˆ ูŠุชูƒูˆู† ููŠู‡ุง Heniz bodies ..

ูˆู„ุงุฒู… ู†ุจุชุนุฏ ุนู† ุงู„ุนูˆุงู…ู„ ุงู„ู„ูŠ ุจุชุฒูŠุฏ ุงู„ุชูƒุณุฑ .

#ุจุงุทู†ุฉ
#ุฃุทูุงู„
๐ŸŸขIn sickle cell disease,

The causative organism of osteomyelitis is Salmonella ,

ุนู„ู‰ ุงู„ุฑุบู… ุงู†ูˆ ุงู„ุดุฎุต ุงู„ุทุจูŠุนูŠ:

The causative organism for osteomyelitis is Staphylococcus aureus

#ุจุงุทู†ุฉ
๐Ÿ›‘ Acute lymphoblastic leukemia ( ALL)


ู…ู† ุงุณู…ู‡ุง :-
๐Ÿ”บ1) SmALL >>> ุญุฌู…ู‡ุง ุตุบูŠุฑ

ูˆู„ุฃู† ุญุฌู…ู‡ุง ุตุบูŠุฑ :-
๐Ÿ”บ2) more in Children ( 1-5 ) years

๐Ÿ”บ3) More infiltration and metastasis to other Tissue like Brain and testis

- more HSM , Lymphadenopathy
- may do Salivary gland swellings


ูˆุจุงู„ุชุงู„ูŠ ูŠุตูŠุฑ ุนู„ุงุฌู‡ุง
๐Ÿ”บ4) Needs intensive treatment for long time , with high recurrence rate .

( induction - consalidation- Mantinance)

ูˆู„ุงู†ู‡ุง ุตุบูŠุฑุฉ ูˆุชุนุจุฑ Pass

๐Ÿ”บ5) Stain with PAS stain .

#ุจุงุทู†ุฉ
ุจุงู„ู†ุณุจุฉ ู„ู„ Acute leukemia ุจุดูƒู„ ุนุงู…

๐Ÿ”ธ infiltrations is more with ALL due to it's small size ; ( HSM , lymphadenopathy, Meningitis, salivary glands " parotid " )


๐Ÿ”ถ Special for AML :
According to FAB :-

M3 [ acute promylocytic Leukemia ] >>> Auer rods >> responds to ATRA >> Good Prognosis

>> DIC may occur in stage M3

>> infiltrations of the Gum [ Gum hypertrophy] in M4 , M5

#ุจุงุทู†ุฉ
Surgical notes
๐Ÿ›‘ Acute lymphoblastic leukemia ( ALL) ู…ู† ุงุณู…ู‡ุง :- ๐Ÿ”บ1) SmALL >>> ุญุฌู…ู‡ุง ุตุบูŠุฑ ูˆู„ุฃู† ุญุฌู…ู‡ุง ุตุบูŠุฑ :- ๐Ÿ”บ2) more in Children ( 1-5 ) years ๐Ÿ”บ3) More infiltration and metastasis to other Tissue like Brain and testis - more HSM , Lymphadenopathy - may do Salivaryโ€ฆ
๐Ÿ”นTherefore ;

In the treatments of ALL ,

ู†ุถูŠู ู„ู‡
Intrathecal methotrexate

ูˆู‡ุฐุง ุฒูŠ ุงู„ู„ูŠ ุนุทูˆู‡ ู„ู„ุฃุทูุงู„ ุงู„ู„ูŠ ุจู…ุณุชุดูู‰ ุงู„ูƒูˆูŠุช ๐Ÿค

#ุจุงุทู†ุฉ
#ุฃุทูุงู„
๐Ÿ›‘ ูˆุฌูˆุฏ ุงู„ Philadelphia chromosome :-
BCR -ABL

ุงูƒุชุดููˆุง ู…ุคุฎุฑุง ููŠ ุฌุงู…ุนุฉ ููŠ ููŠู„ูŠุฏู„ููŠุง ุงู†ูˆ:-

The BCR gene is normally on chromosome number 22.

The ABL gene is normally on chromosome number 9.

The BCR-ABL mutation happens when pieces of BCR and ABL genes break off and switch places.

ูŠุญุตู„ Translocation ูˆูŠุชุจุงุฏู„ูˆุง .


This gene then produces the BCR-ABL protein, which is the type of protein called a tyrosine kinase.


This protein causes CML cells to grow and divide out of control.
ูˆู…ุง ูŠุญุตู„ุด ู„ู‡ุง Apoptosis .

ูˆ ู„ุญุณู† ุงู„ุญุธ ุงูƒุชุดููˆุง ู„ู‡ ุนู„ุงุฌ :-
Tyrosine Kinase inhibitors.

ู…ุซู„:-
imatinib , axitinib , nilotinib

ูˆุบูŠุฑู‡ุง ...

ุงูƒุชุดุงูู‡ู… ู„ูˆุฌูˆุฏ ุงู„ุฌูŠู† ูˆ ุงู„ุนู„ุงุฌ ุงู„ู„ูŠ ูŠูˆู‚ู ุนู…ู„ ุงู„ Tyrosine Kinase ูŠุนุชุจุฑ Good Prognosis ู„ู„ู…ุฑุถู‰ ุงู„ู„ูŠ ุนู†ุฏู‡ู… CML
ู„ุงู†ูˆ ู†ู‚ุฏุฑ ู†ุณูŠุทุฑ ุนู„ู‰ ุงู„ู…ุฑุถ .

โ˜น๏ธโ˜น๏ธโ˜น๏ธโ˜น๏ธ ู„ูƒู† !!!

ู„ูˆ ูƒุงู†ุช AML ุงูˆ ALL ูˆ ุญุตู„ูˆุง ููŠู‡ุง ุงู„ Philadelphia chromosome ู‡ุฐุง ูŠุนุชุจุฑ Bad prognosis !!!!

ู„ูŠุด. ุŸ!
ู„ุงู†ูˆ ูˆุฌูˆุฏู‡ ู…ุนู†ุงุชู‡ ุงู†ู‡ ูƒุงู†ุช Chronic Myloid luekemia.
ูˆ ุญุตู„ Acceleration ูˆ ุจุนุฏู‡ุง Acute leukemia on top of Chronic

ูˆุจู‡ุฐุง ูƒุงู†ุช ุฒู…ุงู† ุชุณุชุฌูŠุจ ู„ู„ุนู„ุงุฌ ูˆุงู„ุงู† ุตุงุฑุช ู…ุง ุชุณุชุฌูŠุจ... ูˆู„ุงุฒู… ู†ุญูˆู„ู‡ุง ู„ุนู„ุงุฌุงุช ุงู„ Acute ุจุงู„ุงุถุงูุฉ ุงู„ู‰ Short Life Expectancy

#ุจุงุทู†ุฉ
๐Ÿ›‘ Splenectomy:-

ู†ุญุชุงุฌู‡ ููŠ ุงู„ุญุงู„ุงุช ุงู„ู„ูŠ ูŠุญุตู„ ููŠู‡ุง Spleenomegaly.

ู„ูƒู† !
ุณุจุจ ุงู„ Spleenomegaly ูŠุง ุงู…ุง ูŠูƒูˆู† ;

- Destruction of cells.
or
- Extramedullary Hematopoisis.

๐ŸŸก๐ŸŸก ู…ู†ุทู‚ูŠุงุง ู„ูˆ ูƒุงู† ุนู†ุฏูŠ Cell Abnormal ุจูŠูƒูˆู† ุงู„ Spleen ูŠูƒุณุฑ ุงู„ุฎู„ุงูŠุง .. ูุงุนู…ู„ Splenectomy ุนุดุงู† ูŠู‚ู„ ุงู„ุชูƒุณูŠุฑ ูˆุงุญุณู† ุงู„ูˆุถุน .
Indications of spleenectomy:-

1- Beta Thalassemia

2- Hereditary spherocytosis or Elliptiocytosis

3- Autoimmune Hemolytic anemia , Autoimmune Thrombocytopenia ( Evan's syndrome )

4- Idiopathic thrombocytopenia

5- Pancytopenia secondary to Hypersplenism ( ex: Malaria )

6- To improve Anemia and Thrombocytopenia in CLL or Accelerated phase of CML



๐ŸŸก๐ŸŸก ุจุงู„ู…ู‚ุงุจู„ ู„ูˆ ูƒุงู† ุนู†ุฏูŠ Bone marrow failure + Spleenomegaly.

ุงู„ู…ูุฑูˆุถ ู…ุง ุงุนู…ู„ุด Spleenectomy ู…ุซู„ :-

1- Aplastic anemia
2- Megaloblastic anemia
3- Mylofibrosis
4- ..


#ุจุงุทู†ุฉ
๐Ÿ”ต Anemia and Thrombocytopenia ??

ููŠ ุงู„ CML , CLL

๐Ÿ”น CML :-
Due to Hypersplenism

๐Ÿ”น CLL
Due to Autoimmune Hemolytic anemia and Thrombocytopenia.
( Evan's syndrome) .

#ุจุงุทู†ุฉ
๐Ÿ”นClinical Staging of CLL

#ุจุงุทู†ุฉ
(ูŠูŽุง ุฃูŽูŠูŽู‘ุชูู‡ูŽุง ุงู„ู†ูŽู‘ูู’ุณู ุงู„ู’ู…ูุทู’ู…ูŽุฆูู†ูŽู‘ุฉู ุงุฑู’ุฌูุนููŠ ุฅูู„ูŽู‰ ุฑูŽุจูู‘ูƒู ุฑูŽุงุถููŠูŽุฉู‹ ู…ูŽู‘ุฑู’ุถููŠูŽู‘ุฉู‹ ููŽุงุฏู’ุฎูู„ููŠ ูููŠ ุนูุจูŽุงุฏููŠ ูˆูŽุงุฏู’ุฎูู„ููŠ ุฌูŽู†ูŽู‘ุชููŠ.).
ุตุฏู‚ ุงู„ู„ู‡ ุงู„ุนุธูŠู…

ุจู‚ู„ูˆุจ ู…ุคู…ู†ุฉ ุจู‚ุถุงุก ุงู„ู„ู‡ ูˆู‚ุฏุฑู‡ ุชู„ู‚ูŠู†ุง ุฌุฏ ุฒู…ูŠู„ุชู†ุง ู…ู‡ุง ุงู„ุฃุบุจุฑูŠ ๐Ÿ˜ญ๐Ÿ’”

ุณุงุฆู„ูŠู† ุงู„ู…ูˆู„ู‰ ุนุฒูˆุฌู„ ุฃู† ูŠุชุบู…ุฏู‡ ุจูˆุงุณุน ุฑุญู…ุชู‡ ุŒ ูˆูŠุณูƒู†ู‡ ูุณูŠุญ ุฌู†ุงุชู‡ ุŒ ูˆูŠู„ู‡ู… ุฃู‡ู„ู‡ ุŒ ูˆุฐูˆูŠู‡ ุงู„ุตุจุฑ ูˆุงู„ุณู„ูˆุงู† ูˆุงู†ุง ู„ู„ู‡ ูˆุงู†ุง ุงู„ูŠู‡ ุฑุงุฌุนูˆู† ุญุณุจู†ุง ุงู„ู„ู‡ ูˆู†ุนู… ุงู„ูˆูƒูŠู„
ุนุธู… ุงู„ู„ู‡ ุงุฌุฑูƒู… ูˆุนุตู… ู‚ู„ูˆุจูƒู… ูˆุงู„ู‡ู…ูƒู… ุงู„ุตุจุฑ ูˆุงู„ุณู„ูˆุงู† ุŒ ุงู„ุจู‚ูŠู‡ ููŠ ุญูŠุงุชูƒู… ุŒ ุฅู†ุง ู„ู„ู‡ ูˆุงู†ุง ุงู„ูŠู‡ ุฑุงุฌุนูˆู†.
(ุงู„ูุงุชุญู‡) ุงู„ู‰ ุฑูˆุญ ุงู„ู…ุฑุญูˆู… ูˆุงู„ู‰ ุฑูˆุญ ู…ูˆุชุงู†ุง ูˆู…ูˆุชุงูƒู….
Assessment of COPD severity and management
#ุจุงุทู†ุฉ