Leukostasis vs Tumor Lysis Syndrome
Leukostasis:
• Pathophysiology: Large, immature blasts and high WBC count cause hyperviscosity syndrome
• Triggers: Diuretics, PRBC transfusion, Dehydration
• Symptoms:
- Neuro: AMS, dizziness, tinnitus, visual changes, stroke symptoms, coma
- Pulm: Hypoxia, dyspnea, +/- CXR infiltrates
- 80% have fever
- ACS, AKI, acute limb ischemia, bowel infarction or priapism
• Lab values: WBC >100k, + lab signs of end organ damage
• Treatment:
- If febrile, give abx
- Aggressive hydration
- Cytoreduction with hydroxyurea +/- TKIs and/or leukapheresis
- TLS prophylaxis with allopurinol +/- rasburicase
Tumor Lysis Syndrome:
• Pathophysiology: Lysis of tumor cells releasing intracellular contents into serum
• Triggers:
- Cytotoxic chemotherapy
- Spontaneous if high proliferation rate or large tumor burden
• Symptoms: N/V, lethargy, hematuria, cardiac dysrhythmias, seizures, tetany/cramps, syncope, sudden death
• Lab values: Elevated K, Elevated phos, Elevated uric acid, Elevated Cr, Low calcium
• Treatment:
- Aggressive hydration
- Hyperkalemia treatment (no calcium gluconate unless worrisome EKG features)
- Non calcium phos binders
- Rasburicase
- Avoid calcium repletion unless symptomatic with tetany or cardiac arrhythmis
SCVMC Internal Medicine @SCVMCMed
#Leukostasis #Tumor #Lysis #Syndrome #TLS #diagnosis #management #hematology
Guillain-Barre Syndrome - Summary
Acute AIDP that presents with rapidly progressive flaccid weakness
Epidemiology:
• 1-2 cases/100,000 per year
• Slightly greater in males than females
Guillain-Barre Pathophysiology:
• Immune response preceding infection
- Campylobacter jejuni
- HIV, CMV, EBV
- Influenza like illnesses, COVID 19
- Zika virus
- Vaccination (flu, meningococcal, H1N1)
Clinical Manifestations:
• Fairly symmetric muscle weakness
• Absent or depressed DTR’s
• Onset: Few days to a week
• Weakness: mild difficulty with walking to nearly complete paralysis of all extremity, facial, respiratory and bulbar muscles
Guillain-Barre Clinical Features:
• Weakness: Starts in legs and ascends usually. Can begin in arms or face (10% of patients)
• Respiratory Muscle Weakness: May require ventilator support (10-30%)
• Facial Nerve Palsies / Oropharyngeal Weakness
• Oculomotor Weakness
• Decreased / Absent Reflexes: Arms or legs
• Paresthesias: Hands or feet
• Pain: Due to nerve root inflammation. Back or extremities
• Dysautonomia: Life threatening labile BP and arrythmias
• Other: Diarrhea/constipation, hyponatremia, bradycardia, urinary retention, tachycardia, reversible cardiomyopathy, Horner syndrome, Sudden death
Miller Fisher Syndrome:
• Opthaloplegia
• Ataxia
• Areflexia
Guillain-Barre Diagnosis:
• CSF:
- CSF PROTEIN
- NORMAL CSF WBC (Albuminocytologic dissociation)
• Electrodiagnostic studies - EMG, NCV can classify the main variants:
(1) acute polyneuropathy with demyelination features
(2) axonal form
• MRI - Thickening and enhancement of intrathecal spinal nerve roots and cauda equina
• GQ1b IgG Antibody associated with Miller Fisher Variant.
Treatment:
• Plasmapheresis + IVIG
• Mechanical ventilation
• Steroids contraindicated- worse outcome
#GuillainBarre #Guillain #Barre #Syndrome #diagnosis #management #treatment #neurology
Chronic Lymphocytic Leukemia (CLL) - Summary
Overview:
• Most common leukemia in adults
• Disorder of morphologically mature B Lymphocytes
• Median survival 4-6 years but >10 years in patients with minimal tumor burden
Diagnosis:
• Peripheral smear → small rnature-appearing lymphocytes, smudge cells are characteristic
• Peripheral flow cytometry → >500/uL clonal B lymphocytes with typical immunophenotype
Clinical Manifestations:
1) Incidental detection of Vmphocytosis on routine CBC (rnost common)
2) Asymptomatic lymphadenopathy
3) B symptoms → fevers, night sweats, weight loss
4) Splenomegaly
5) Symptoms of anemia or thrombocytopenia
Complications:
1) Infection due to hypogammaglobulinemia
2) Autoimmune cytopenias (ITP, AIHA, PRCA, autoimmune neutropenia)
3) Transformation to large cell lymphoma (Richter transformation)
Treatment:
• "Watchful Waiting" →several RCTs have found no benefit of immediate chemotherapy for low-risk disease
• Indications for Treatment:
- Constitutional symptoms
- Symptomatic massive lymphadenopathy and/or splenomegaly
- Progressive bore marrow failure with anemia or thrombocytopenia
- Lymphocyte doubling time < 6 months
- Autoimmune cytopenias (ITP. AIHA, PRCA)
• Treatment Agents:
1) Alkylating agents (bendamustine, chlorambucil)
2) Purine analogs (fludarabine)
3) Monoclonal antibodies (rituximab, obinutuzumab)
4) Ibrutinib (Bruton kinase inhibitor)
5) Venetoclax (BCL2 inhibitor)
by Lauren Banaszak, MD @LaurenBzak via @uw_IMresidency
#CLL #Chronic #Lymphocytic #Leukemia #oncology #hematology #hemeonc #diagnosis #management
Differentiation Syndrome in APML
Epidemiology:
• Incidence: common in APL (2-48% depending on the study)
• Triggers: ATRA treatment
Pathophysiology:
• Cytokine Release from blast cells → SIRS response
• Cathepsin G release → vascular permeability and endothelial damage
• Induce blast cell adhesion and endothelial damage
Prophylaxis:
• Attempt if high risk if WBC > 5 or elevated Cr
• Prednisone 5mg/kg/day vs. 2.5 mg/m2
Presentation: Subacute-Acute:
• Common: fever, myalgias, HoTN, edema and effusions, weight gain. More common in severe
• Rare: DAH, acute febrile neutrophilic dermatosis
Diagnosis:
• Labs: leukocytosis and coagulopathy common
• Imaging: CXR- pulmonary opacities
• Diagnosis: >3 symptoms, or> 1 with no other explanation. Moderate > 2/3 and severe >4. Timing, generally either within 6 days or 15 days of ATRA initiation
• Differential Diagnosis: Infection (sepsis), PE, DAH, CHF, Anaphylaxis, Acute Renal Failure
Treatment:
• Steroids: IV Dexamethasone 10 mg q12h -> 10 mg q6h
• Cytoreductive: hydroxycarbamide 500 mg QD until normal WBC
• Continue ATRA unless severe APLS, organ dysfuxtion, ICU, refractory to steroids
• Supportive Care: Diuresis for fluid overload, PCC to reverse coagulopathy, RRT/IMV as needed
- Dr. Noah Rosenberg @nsrosenberg
#Differentiation #Syndrome #APML #diagnosis #management #hematology #oncology
Stiff Person Syndrome (SPS) - Diagnosis and Management Summary
Epidemiology:
• Prevalence: 1-2 cases per million - rare
• Age 20-50, Females 2-3 times than men
• Commonly associated with DM-1, thyroiditis, vitiligo, and pernicious anemia
Stiff Person Syndrome Pathophysiology:
• Associated with autoimmunity
• Research indicates that it is the result of an autoimmune response gone awry in the brain and spinal cord.
Clinical Manifestations of Stiff Person Syndrome:
• Progressive muscle stiffness, rigidity, spasm - axial muscles
• Severely impaired ambulation
• Excessive muscle rigidity - lumbar, trunk, proximal muscles - sustained muscle contractions (agonist and antagonist muscles)
• Wide based gait with tendency to fall
• Triggers - sudden movement, noise, emotional upset
• Spasms - visualized-tight, rock-hard, board-like feel
• Autonomic dysfunction - hyperpyrexia, diaphoresis, high HR
• Sudden death - diaphragm spasm - apnea
Stiff Person Syndrome - Diagnosis:
• High index of suspicion
• Presence of:
- Stiffness in axial and limb muscles resulting in impairment of ambulation
- Presence of superimposed spasms triggered by noise, movement or emotions
- A positive therapeutic response to diazepam or findings of continuous motor unit activity on EMG
- Absence of other neurological disorders
• Testing anti-GAD (positive in 2/3 of pts)
• Therapeutic trial of diazepam
• EMG testing
Lab Findings in Stiff Person Syndrome:
• Anti-GAD Ab (>1000u/ml)
- People with SPS have elevated levels of GAD, an antibody that works against an enzyme involved in the synthesis of an important neurotransmitter in the brain.
• CSF: Oligoclonal bands
• Normal ESR, CRP
• Other autoantibodies - TPO, TTG, etc
EMG Studies:
• Continuous motor-unit activity, typically decreased by diazepam, sleep
DDx:
• Neuromyotonia, Tetanus, Strychnine poisoning, Parkinson's disease, Multiple sclerosis, Ankylosing spondylitis, Axial dystonia, Focal limb or joint disorders, Fibromyalgia, Psychosomatic illness, Anxiety, Phobia
Stiff Person Syndrome Treatment:
• Initial/Symptomatic Therapy:
- BDZ: Diazepam or clonazepam
• Resistant to Initial Symptomatic Therapy:
- Baclofen
• Severe Disease/Resistant:
- IVIG
- If Resistant to IVIG: Rituximab (anti-CD20), plasma exchange, or others (botox, sodium valproate, vigabatrin, propofol)
#Stiff #Person #Syndrome #SPS #Diagnosis #Management #treatment #neurology
Orthopedic Emergencies
Compartment Syndrome
• An increase in compartment pressure to the point where tissue perfusion is impaired.
• Initial Management
- Remove all circumferential dressings/casts
- Ensure leg is at level of the heart - the affected part should not be elevated above the level of the heart because this maneuver does not improve venous outflow and reduces arterial inflow
- Remove any traction
• Definitive management:
- Compartment fasciotomy-2 incisions, 15 cm long
- Delay>12 hr. often results in irreversible muscle and nerve damage in that compartment
Fat Embolism Syndrome
• Syndrome caused by presence of fat globules in the lung parenchyma and peripheral circulation. Usually subclinical event after long bone fractures in young adults (tibia/fibula) and hip fractures in elderly. Syndrome usually appear in 1-2 days after an acute injury or after IM nailing.
• Management: 1. Oxygenation 2. Fluid resuscitation 3. Surgical Care - early stabilization of long bone fractures
Necrotizing Fasciitis
Rapidly progressive inflammatory infection of the fascia, with secondary necrosis of
the subcutaneous tissues. The speed of spread is directly proportional to the thickness
of the subcutaneous layer. Necrotizing fasciitis moves along the fascial plane.
• Diagnosis: requires a high degree of suspicion
- H/O antecedent trauma or surgery
- Intense pain over the involved skin and underlying muscle; over the next several hours to days, the local pain progresses to anaesthesia.
- Fever, malaise, and myalgia
- Edema extending beyond the area of erythema, skin vesicles, and crepitus.
- Comorbid factors, including DM
• Treatment
- Prompt surgical debridement is continued until tissue necrosis ceases and the growth of fresh viable tissue is observed.
- Antibiotic (broad spectrum covering both gram positive and negative)
- Hyperbaric oxygen therapy (HBOT)
Spinal Shock
• Temporary loss of spinal cord function and reflex activity below the level of spinal cord injury, characterised by bradycardia, hypotension (due to loss of sympathethic tone), and an absent bulbocarvenosus reflex
• Spinal shock vs Neurogenic Shock vs Hypovolemic Shock
#Orthopedics #Emergencies #diagnosis #management #treatment
Hypoxemia Differential Diagnosis Algorithm by A-a Gradient
Normal A-a Gradient:
• ↓ Atmospheric pressure
• Hypoventilation: CNS depression, neuromuscular disease, ↓ chest wall compliance
↑ A-a Gradient:
• Right-to-Left Shunt: Cardiac, Vascular, Alveolar
• V/Q Mismatch: ↓Ventilation (↑Shunting), ↓Perfusion (↑Dead space)
• ↓Diffusion Capacity
Matthew Ho, MD PhD @MatthewHoMD
#Hypoxemia #Hypoxia #Differential #Diagnosis #Algorithm #pulmonary #Aa #Gradient
