Lincoln Shared "pathoma" - 134 Picmonics
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pathoma
Leukocyte Adhesion Deficiency
- Pathophysiology
- Autosomal Recessive
- Integrin CD18 Protein
- Defect in LFA-1
- Attached to ICAM
- Signs and Symptoms
- Neutrophilia
- Bacterial Infections
- Non-Pyogenic
- Delayed Separation of Umbilical Cord
2 mins
Chediak-Higashi Syndrome
- Pathophysiology
- Autosomal Recessive
- Defective Lysosomal Trafficking Regulator Gene (LYST)
- Defect In Microtubular Function
- Decrease In Phagocytosis
- Signs And Symptoms
- Recurrent Pyogenic Infections
- Staph Aureus And Streptococci
- Partial Albinism
- Peripheral Neuropathy
- Pancytopenia
- Diagnosis
- Giant Granules In Granulocytes And Platelets
2 mins
Chronic Granulomatous Disease
- Pathophysiology
- Most Commonly X-linked Recessive
- Defective NADPH Oxidase (Enzyme Complex)
- Decreased Hydrogen Peroxide
- Impaired Intracellular Killing in Phagocytes
- Signs and Symptoms
- Recurrent Purulent Skin and Lung Infections
- Catalase Positive Organisms
- Diagnosis
- Negative Nitroblue Tetrazolium Test
- DHR (Dihydrorhodamine) Flow Cytometry
- Treatment
- Antimicrobial Prophylaxis
- TMP-SMX
- Itraconazole
- Interferon-Gamma
3 mins
Thymic Aplasia (DiGeorge Syndrome)
- Pathophysiology
- DiGeorge Syndrome
- 22q11 Deletion
- Failure to Develop Third and Fourth Pharyngeal Pouches
- Signs and Symptoms
- Undeveloped Thymus
- T Cell Deficiency
- Recurrent Viral and Fungal Infections
- Undeveloped Parathyroids
- Hypocalcemia
- Tetany
- Aortic Defects
- Congenital Heart Defects
- Diagnosis
- Absent Thymic Shadow on CXR
2 mins
Severe Combined Immunodeficiency (SCID)
- Both B and T Cell Deficiency
- Recurrent Viral, Bacterial, Fungal, Protozoal Infections
- Defective Interleukin (IL) 2 Receptor
- X-Linked
- Adenosine Deaminase Deficiency
- Failure to Synthesize MHC II Antigens
- Treat with Bone Marrow Transplant
2 mins
Bruton's Agammaglobulinemia
- Pathophysiology
- X-linked Recessive
- Defective Bruton Tyrosine Kinase
- Blocks B Cell Maturation
- Signs & Symptoms
- Decreased Immunoglobulins of All Classes
- Bacterial Infections After Six Months
- Maternal IgG Protects Before Six Months
2 mins
Hyper IgM Syndrome
- Pathophysiology
- Deficient CD40 Ligand on Helper T-cells
- Defective CD40 Receptor on B-cells
- Inability to Class Switch
- Signs and Symptoms
- Pyogenic Abscess
- Decreased IgG, IgA, IgE
4 mins
Wiskott Aldrich
- Pathophysiology
- X-linked Recessive
- WASp Protein Mutation
- Antigen Presentation Defect
- Progressive Deletion of B and T Cells
- Signs and Symptoms
- Thrombocytopenia
- Eczema
- Infections
- Decreased IgM
- Increased IgE, IgA
2 mins
Systemic Lupus Erythematosus (SLE) Diagnosis and Clinical Features
- IM DAMN SHARP acronym
- Immunoglobulins
- Malar rash
- Discoid rash
- Antinuclear antibody
- Mucositis
- Neurologic disorders
- Serositis
- Hematologic disorders
- Arthritis
- Renal disorders
- Photosensitivity
2 mins
Sjögren’s Syndrome
- Mechanism
- Autoimmune Exocrine Gland Destruction
- Symptoms (Classic Triad)
- Xerostomia
- Xerophthalmia
- Arthritis
- Diagnosis
- Anti SS-B (La)
- Anti SS-A (Ro)
- Schirmer's Test
- Salivary Gland Biopsy
- Treatment
- Symptomatic Treatment
- Complications
- Lymphoma
- Other
- Sicca Syndrome
4 mins
Bernard-Soulier Syndrome
- Mechanism
- Autosomal Recessive
- Deficiency of Glycoprotein Ib (GpIb) Receptor
- Symptoms
- Mucosal Membrane Bleeding
- Menorrhagia
- Easy Bruising
- Labs
- Large Platelets
- Increased Bleeding Time
- No Platelet Agglutination with Ristocetin
3 mins
Von Willebrand Disease
- Mixed platelet and coagulation disorder
- Mechanism
- Autosomal Dominant Condition Leading to Reduced vWF
- Defect in platelet plug formation
- Labs
- Increased Bleeding Time
- PTT increased
- Factor VIII decreased
- Normal platelet count
- Treatment
- DDAVP (synthetic vasopressin)
1 min
Disseminated Intravascular Coagulation (DIC)
- Pathophysiology
- Bleeding State
- Activation of Clotting Factors
- Deficiency of Clotting Factors
- Causes
- Sepsis
- Trauma
- Obstetric Complications
- Acute Pancreatitis
- Malignancy
- Nephrotic Syndrome
- Transfusion
2 mins
Plummer-Vinson Syndrome
- Triad of Symptoms
- Dysphagia
- Esophageal Webs
- Glossitis
- Iron Deficiency Anemia
- Association
- Squamous Cell Cancer of Esophagus
1 min
Hereditary Spherocytosis Disease
- Northern Europeans
- Mechanisms
- Autosomal Dominant
- Spectrin/Ankyrin Deficiency
- Spherocyte Formation
- Hemolytic Anemia
- Symptoms
- Bilirubin Gallstones
- Jaundice
- Splenomegaly
3 mins
Sickle Cell Anemia (Signs and Complications)
- Signs
- Crew-cut on Skull X-ray
- Complications
- Auto-splenectomy
- Painful Crisis
- Aplastic Crisis
- Splenic-sequestration Crisis
- Hyper-hemolytic Syndrome
- Renal Papillary Necrosis
- Salmonella osteomyelitis
- Pulmonary Hypertension
- Hyposthenuria
2 mins
Paroxysmal Nocturnal Hemoglobinuria (PNH)
- Pathophysiology
- Defective PIG-A Gene
- Deficiency of DAF (Decay Accelerating Factor CD55)
- Symptoms
- Dark Urine in the Morning
- Intravascular Hemolysis
- Thrombosis
- Diagnosis
- Decreased CD55 and CD59
- Treatment
- RBC Transfusion
- Eculizumab
- Bone Marrow Transplant is Curative
3 mins
Epstein Barr Virus Mononucleosis
- Mononucleosis
- Fever
- Hepatomegaly
- Splenomegaly
- Pharyngitis
- Posterior Cervical Lymphadenopathy
- Atypical Lymphocytes
- Heterophile Antibodies
- Rash Develops if treated with Ampicillin
- Hodgkin's Lymphoma
2 mins
Acute Lymphoblastic Leukemia (ALL)
- Ages < 15
- May Present with Bone Marrow Involvement in Children
- Mediastinal Mass in Adolescents
- TdT Positive
- CALLA Positive
- t(12;21) Better Prognosis
- Spread to CNS and Testes
- Most Responsive to Chemotherapy
2 mins
Acute Myelogenous Leukemia (AML)
- Myeloblasts Increase on Peripheral Smear
- Median Onset 60 Years
- T 15;17 M3 Subtype
- Auer Rods
- Disseminated Intravascular Coagulation (DIC) is a Common Presentation
- M5 Gum Infiltration
- Responds to Vitamin A
3 mins
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