UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
Romanowsky staining or Romanowsky–Giemsa staining, is a prototypical staining technique, widely used in hematology and cytopathology.
They are used to differentiate cells for microscopic examination in air dried cytological smears or pathological specimens, especially blood and bone marrow films, and to detect parasites such as malaria within the blood.
Romanowsky stains is a neutral dye containing both acid and basic dyes in combination. It contains both azure B (electron acceptor) and eosin Y (electron donor).
The value of Romanowsky staining lies in its ability to produce a wide range of hues, allowing cellular components to be easily differentiated. This phenomenon is referred to as the Romanowsky effect, or more generally as metachromasia.
These stains allow better estimation of cell size, nuclear size, cell cytoplasm and identify ground substances by metachromasia.
I have listed out the LE cells structure and Microscopical examinaton of LE CELLS, Difference between tart cells and le cells, clinical symptoms and diagnostic procedure.
challenges in interpreting abnormal hemoglobin study- the key is to correlate with patient age, ethnicity,RBC indices & morphology findings. Two tier approach for correct characterization of abnormal hemoglobins of HPLC &/or capillary electrophoresis.
Romanowsky staining or Romanowsky–Giemsa staining, is a prototypical staining technique, widely used in hematology and cytopathology.
They are used to differentiate cells for microscopic examination in air dried cytological smears or pathological specimens, especially blood and bone marrow films, and to detect parasites such as malaria within the blood.
Romanowsky stains is a neutral dye containing both acid and basic dyes in combination. It contains both azure B (electron acceptor) and eosin Y (electron donor).
The value of Romanowsky staining lies in its ability to produce a wide range of hues, allowing cellular components to be easily differentiated. This phenomenon is referred to as the Romanowsky effect, or more generally as metachromasia.
These stains allow better estimation of cell size, nuclear size, cell cytoplasm and identify ground substances by metachromasia.
I have listed out the LE cells structure and Microscopical examinaton of LE CELLS, Difference between tart cells and le cells, clinical symptoms and diagnostic procedure.
challenges in interpreting abnormal hemoglobin study- the key is to correlate with patient age, ethnicity,RBC indices & morphology findings. Two tier approach for correct characterization of abnormal hemoglobins of HPLC &/or capillary electrophoresis.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
My son had Wiskott Aldrich Syndrome (WAS). He had a bone marrow transplant in August 2006. His WAS is healed. This presentation was designed by some grad students. Some of the content is from my blog and it pictures my son, David. http://www.davidmcnally.blogspot.com
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
Blood banking and transfusion medicine i&iiAbdulKaderSouid
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
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Pulmonary Thromboembolism - etilogy, types, medical- Surgical and nursing man...VarunMahajani
Disruption of blood supply to lung alveoli due to blockage of one or more pulmonary blood vessels is called as Pulmonary thromboembolism. In this presentation we will discuss its causes, types and its management in depth.
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
The prostate is an exocrine gland of the male mammalian reproductive system
It is a walnut-sized gland that forms part of the male reproductive system and is located in front of the rectum and just below the urinary bladder
Function is to store and secrete a clear, slightly alkaline fluid that constitutes 10-30% of the volume of the seminal fluid that along with the spermatozoa, constitutes semen
A healthy human prostate measures (4cm-vertical, by 3cm-horizontal, 2cm ant-post ).
It surrounds the urethra just below the urinary bladder. It has anterior, median, posterior and two lateral lobes
It’s work is regulated by androgens which are responsible for male sex characteristics
Generalised disease of the prostate due to hormonal derangement which leads to non malignant enlargement of the gland (increase in the number of epithelial cells and stromal tissue)to cause compression of the urethra leading to symptoms (LUTS
Prix Galien International 2024 Forum ProgramLevi Shapiro
June 20, 2024, Prix Galien International and Jerusalem Ethics Forum in ROME. Detailed agenda including panels:
- ADVANCES IN CARDIOLOGY: A NEW PARADIGM IS COMING
- WOMEN’S HEALTH: FERTILITY PRESERVATION
- WHAT’S NEW IN THE TREATMENT OF INFECTIOUS,
ONCOLOGICAL AND INFLAMMATORY SKIN DISEASES?
- ARTIFICIAL INTELLIGENCE AND ETHICS
- GENE THERAPY
- BEYOND BORDERS: GLOBAL INITIATIVES FOR DEMOCRATIZING LIFE SCIENCE TECHNOLOGIES AND PROMOTING ACCESS TO HEALTHCARE
- ETHICAL CHALLENGES IN LIFE SCIENCES
- Prix Galien International Awards Ceremony
Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
Couples presenting to the infertility clinic- Do they really have infertility...Sujoy Dasgupta
Dr Sujoy Dasgupta presented the study on "Couples presenting to the infertility clinic- Do they really have infertility? – The unexplored stories of non-consummation" in the 13th Congress of the Asia Pacific Initiative on Reproduction (ASPIRE 2024) at Manila on 24 May, 2024.
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
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Disorders of myelopoiesis.ppt
1. Abdul-Kader Souid
↓ Neutrophil Count (Neutropenia)
• Inherited (bone marrow examination is necessary):
• Neutropenia, severe congenital, 1, AD; SCN1 (MIM#202700); mutations in
ELANE (MIM#130130)
• Cyclic neutropenia, AD (MIM#162800); mutations in ELANE
(MIM#130130)
• Neutropenia, severe congenital, 3, AR; SCN3 (MIM#610738); mutations in
HAX1 (MIM#605998)
• Acquired: Viral infection (common), drug-induced (common), bone marrow
failure syndrome (rare)
↓ Neutrophil Function
• Granulomatous disease, chronic, X-linked (XLR); CDGX (MIM#306400): A
primary immunodeficiency (defective microbial killing); mutations in CYBB
(MIM#300481).
• Hyper-IgE recurrent infection syndrome (HIES), AD (MIM#147060): A
primary immunodeficiency characterized by chronic eczema, recurrent
Staphylococcal infections, ↑serum IgE, and eosinophilia due to STAT3
(MIM#102582) mutations.
Disorders of MyelopoiesisMust know
AD, autosomal dominant; AR, autosomal recessive; XLR, X-linked recessive
2. Myelopoiesis
• Hematopoiesis: Hematopoietic multipotent (stem) cells can differentiate to →
erythrocytes, megakaryocytes, neutrophils, eosinophils, basophils, monocytes,
T lymphocytes, B lymphocytes, natural killer cells, dendritic cells, and mast cells.
• Myelopoiesis I: Multipotent (stem) cell → CFU-GEMM (CD34+/ HLA-DR+).
• Myelopoiesis II: CFU-GEMM → CFU-GM (CD34+/ HLA-DR+/ CD-64+).
– CFU-GM is driven by the growth factors c-kit ligand, IL-3, IL-6, and GM-CSF.
• Myelopoiesis III: CFU-GM → CFU-G (CD34+/ HLA-DR+/ CD-64+).
– CFU-G is driven by the growth factor G-CSF.
CFU: Colony forming units that grow at 37ºC in semisolid medium as pure colonies in 7 days.
CFU-GEMM: Colony forming units of precursor to granulocytes, erythrocytes, macrophages
(monocytes), and megakaryocytes.
CFU-GM: Colony forming units of precursor to granulocytes and monocytes/macrophages.
CFU-G: Colony forming units of precursor to granulocytes.
GM-CSF: Granulocyte-macrophage colony-stimulating factor.
G-CSF: Granulocyte colony-stimulating factor.
CFU-GEMM CFU-GM CFU-G
Reading
3. Neutrophil Function
(1) Neutrophil entrance from marrow to circulation.
(2) Rolling & tethering (adherence, mediated by selectins).
(3) Adhesion: Activated endothelial cells express
intercellular adhesion molecules (ICAM), which serve
as ligands for neutrophil β2 integrins (impaired in
‘leukocyte adhesion deficiency’, LAD).
(4) Invasion through the vascular basement
membrane with release of proteases and reactive
oxidative intermediates, destructing extracellular
matrix and allowing migration into tissue (impaired
in chronic granulomatous disease).
(5-6) Uptake of pathogens into the phagocytic vacuole with concomitant degranulation both into the
phagocytic vacuole and to the exterior (impaired in Chédiak-Higashi).
(7) Chemokines [IL-8, monocyte chemoattractant protein (MCP)-1, macrophage inflammatory
protein (MIP)-1α, and IL-1β] recruit cells of the immune system (impaired in hyper-IgE syndrome).
(8) Extracellular traps are extrusions of chromatin and cationic bactericidal granule proteins.
Reading
4. Neutrophil Function
Chemotaxis → Motility → Adhesion→ Ingestion → Killing
9/26/2018
Lysis of the cytoplasmic granule (G)
against two ingested zymosan
particles (Z). The dense body of the
granule disappears from view in the
interval of 5 sec. (J Exp Med
1962;116:827)
Taxis = motion or orientation
Zymosan = a glucan of repeating glucose units found on surface of fungi.
This degranulation
process is impaired in
Chédiak-Higashi
disease
Must know
5. Neutrophil “Respiratory Burst”
Most homes have a bottle of hypochlorite (Ocl-) in the kitchen or bathroom
(Domestos, Chlorox, etc.); as advertised, these products kills all household germs.
These reactions are
impaired in chronic
granulomatous disease
(↓killing of catalase-
positive bacteria)
Chronic granulomatous disease → infections with catalase-positive organisms
(Staphylococcus aureus, Serratia marcescens, Acinetobacter anitratus, Salmonella,
Burkholderia cepacia, Aspergillus, Nocardia) → necrotizing granulomas. Initial diagnosis
is made by the oxidative burst flow cytometry test (phagocytes fail to oxidize rhodamine).
Must know
Bacterial Catalase
((catalase-positive bacteria)
H2O + ½O2
6. 6
Hyper-IgE recurrent infection syndrome (HIES), AD (MIM#147060): A primary
immunodeficiency characterized by chronic eczema, recurrent Staphylococcal
infections, ↑ serum IgE, and eosinophilia due to STAT3 (MIM#102582) mutations.
(impaired chemotaxis and cytokine production)
This patient (Malaysian) had severe, persistent atopic dermatitis (intense itching), severe
atopy (asthma and allergic rhinitis), and a recent invasive infection (right hip joint and
bone). One year ago, Staphylococcus aureus and varicella-zoster virus (VZV) infections.
He had a significant history of food allergy, such as crab and shrimps which severely
flare his skin disease.
The father has similar moderate-to-severe atopic dermatitis and allergic rhinitis. His
sister has a much milder atopic dermatitis. His maternal grandfather has asthma and
maternal aunt has atopic dermatitis.
Must know
7. Mutations in LYST (MIM#606897), which encodes a lysosomal
trafficking regulator protein
↓Neutrophil chemotaxis + ↓Degranulation → ↓Bactericidal activity
↓Platelet storage pool → Bleeding
↓Melanosomes → Albinism
7
Union of lysosomal (giant)
granules in Chédiak-Higashi
Albinism
Optional Degranulation Abnormality
Chédiak-Higashi Syndrome (CHS, OMIM#214500, AR)
8. 8
Leukocyte adhesion deficiency, type I; LAD, AR (MIM#116920)
Mutations in ITGB2 (MIM#600065; integrin, beta-2)
(recurrent bacterial infections; impaired pus formation and wound healing)
Lack of CD18/CD11 surface adhesive glycoproteins (β2 integrins) on
neutrophil membranes → neutrophilia + pyogenic infections without
pus.
Infections in a toddler with leukocyte adhesion deficiency
Optional
9. Severe Congenital Neutropenia
• Recurrent otitis, gingivitis, pneumonia, enteritis, and bacteremia in early infancy.
• Neutrophil count is <0.2 ×109/L with monocytosis, anemia, and thrombocytosis.
• Bone marrow shows neutrophil maturation arrest at the myeloblast (no mature
neutrophils).
• Patients are at risk for developing acute myelogenous leukemia (AML).
• G-CSF is effective (↑increasing neutrophil counts & ↓infections).
• Hematopoietic transplantation is the only curative therapy.
9/26/2018 9
Must know
• Neutropenia, severe congenital, 1, AD; SCN1 (MIM#202700); mutations in
ELANE (MIM#130130)
• Neutropenia, severe congenital, 3, AR; SCN3 (MIM#610738); mutations in
HAX1 (MIM#605998)
10. • Recurrent neutropenia, with a cycle period of about 21 days.
• The diagnosis is established by measuring the neutrophil count twice a
week for eight consecutive weeks.
• Supportive care includes regular dental care, prompt antibacterial
therapy as needed, and genetic counseling.
• Granulocyte colony-stimulating factor (G-CSF) is given at the nadir of
the neutrophil counts.
Note: Mutations in the elastase gene are associated with both cyclic
neutropenia and severe congenital neutropenia.
Cyclic neutropenia, AD (MIM#162800); mutations in ELANE (MIM#130130)
Must know
11. • Neutropenia due to proliferative defect and ↑apoptosis of
early myeloid progenitor cells.
• Clinical findings begins in the neonatal period and include short
stature, inadequate weight gain, skeletal abnormalities, infections
(otitis media, sinusitis, pneumonia), and large stools (steatorrhea,
malabsorption, pancreatic exocrine deficiency), and marrow
failure.
– Imaging confirms extensive fatty replacement of the pancreas.
– The presentation resembles cystic fibrosis (sweat chloride is
normal in Shwachman syndrome and high in cystic fibrosis).
• The risk of evolution to myelodysplastic syndrome and acute
myelogenous leukemia is at least 20%.
• G-CSF raises the neutrophil counts.
• Hematopoietic stem cell transplantation is curative.
11
Shwachman-Diamond Syndrome 1; SDS1, AR
(MIM#260400); mutations in SBDS (MIM#607444)
Optional
12. 1. Neutrophil respiratory burst defect + XLR +
catalase-positive pathogens
2. Giant neutrophil granules + albinism
3. Negative CD11/CD18 expression + neutrophilia
4. Eczema + infections + Stat 3 mutation
5. Pancreatic insufficiency + neutropenia + SBDS
mutation
6. Elastase gene (ELANE)
7. Risks of developing AML
8. Treatment with G-CSF
9. Hematopoietic stem cell transplantation
A. Severe congenital neutropenia
B. Shwachman-Diamond Syndrome
C. Cyclic neutropenia
D. Chronic granulomatous disease
E. Chédiak-Higashi syndrome
F. Leukocyte adhesion deficiency
G. Hyper IgE syndrome
Fever with neutropenia is best treated with monotherapy (meropenem, cefepime or
ceftazidime). Although these regimens do not cover methicillin-resistant Staphylococcus
aureus and coagulase-negative staphylococci, vancomycin should be withheld in low-risk
patients pending cultures in an effort to prevent vancomycin-resistant enterococci.
Must Know Pearls
Freifeld AG, et al. Infectious Diseases Society of America. Clinical practice guideline for the use of antimicrobial agents in neutropenic
patients with cancer: 2010 update by the Infectious Diseases Society of America. Clin Infect Dis. 2011;52:e56-93. doi: 10.1093/cid/cir073.
13. Two brothers present to the clinic for evaluation of recurrent neutropenia. They suffer
from regular episodes of mucositis, sinusitis, otitis media, pharyngitis and tonsillitis.
Their maternal grandfather, mother, maternal aunt and her son all have neutropenia.
9/26/2018 13
0
500
1000
1500
2000
0 10 20 30 40 50
ANC
days
CBC on the 5-year-old boy reveals absolute neutrophil
count (ANC) of 300/mL (0.3 x109/L). The results of his
ANC over 45 days are shown. Blood smear shows normal
neutrophil morphology.
Which of the following is the most likely diagnosis?
A. Chédiak-Higashi syndrome
B. Leukocyte adhesion deficiency
C. Hyper IgE syndrome
D. Severe congenital neutropenia
E. Cyclic neutropenia