Hypercoagulable States
What is a risk factor for Factor 5 Leiden mutation?
Caucasian
What vitamin is essential to the coagulation cascade?
Vitamin K
spleen
What are some causes of acquired idiopathic sideroblastic anemias?
Toxins (lead, ethanol, zinc)
Drugs (Isoniazid, pyrazinamide)
What is the main type of Hodgkin's lymphoma?
Classical Hodgkin’s Lymphoma, (cHL)
What medication should be avoided as a choice for oral maintenance anticoagulation in Protein C & S deficiency?
Warfarin
What conidition is referred to as the “Christmas Disease”?
Hemophilia B
What is the pathophysiology of G6PD Deficiency?
Mutations in gene G6PDH → decreased G6PDH activity → decreased NADPH → decreased regeneration of GSH from GSSG by glutathione reductase (uses NADPH) → oxidation due to decreased levels of GSH and increased levels of intracellular oxidants (O2), SH groups of Hb (form Heinz bodies), and membrane proteins
Alters membrane structure and increases susceptibility to ingestion by macrophages (peroxidative damage to lipids in membrane possible) → hemolysis
Symptoms of B12 deficiency include:
• Neurologic symptoms, which can be permanent
• Stocking-glove paresthesia, balance problems, dementia
What is the treatment for classical Hodgkin's lymphoma?
ABVD (Adriamycin [doxorubicin] + Bleomycin+Vinblastine+Dacarbazine) chemotherapy w/ possible addition of limited radiation therapy for those with favorable prognosis
Pregnancy and SLE are strong risk factors for this condition. Patients present with livedo reticularis.
Antiphospholipid syndrome
What is the treatment for a pt presenting with easy bruising, hematomas, GI bleeding, hematuria, and painful hemarthrosis of the knee? Lab findings show a prolonged pTT and normal PT/platelet count.
DDAVP (for mild cases)
Recombinant factor VIII infusions for severe
deficiencies during trauma/surgeries
A 20-y/o AA male presents to the ED with sudden severe pain in his lower back and thighs, which started 6 hours ago. He describes the pain as constant, sharp, and aching. The patient has a hx of similar episodes in the past, especially when under stress or dehydration, which usually resolve with pain medication.
PE: in moderate distress due to pain. No sign of infection or trauma. PE is otherwise unremarkable. What is his treatment?
Labs:
IV morphine or dilaudid, IV hydration, and oxygen
Pt has new weakness in her legs. She has vertebral fracture on X-ray. She denies any trauma recently. Labs reveal increased calcium and increased creatine. You order serum protein electrophoresis. What would you expect to see on the results of the test?
monoclonal IgG spike
Key clinical manifestation of Waldenstrom Macroglobulinemia
Hyperviscosity syndrome
A 32-year-old woman presents with a 3-day history of swelling, pain, and redness in her right calf. She denies trauma but has a family history of blood clots (father with multiple DVTs, maternal aunt with PE). On examination, there is calf swelling, tenderness, and a positive Homan's sign. Ultrasound confirms deep vein thrombosis (DVT). D-dimer is elevated and Antithrombin III activity is low (<60%). What is the diagnosis?
Antithrombin III deficiency
A 24-y/o woman presents with a 2-week history of easy bruising, frequent nosebleeds, and heavy menstrual bleeding. She reports a family hx of similar sx with her mother and maternal uncle. On PE, there are ecchymoses on her arms and legs, and her mucous membranes appear normal with no active bleeding.
Labs: PLT count and PT are normal. aPTT is prolonged, vWF activity is low, and factor VIII activity is low. What is her dx?
von Willebrand disease
A 35-y/o woman presents with a 3-day hx of fatigue, pale skin, and SOB on exertion. She also reports dark-colored urine in the morning and a mild headache. She has no significant PMH, but she has been recently treated for a viral upper respiratory infection.
On exam, she appears pale and slightly jaundiced. Her vital signs are stable. There are no signs of lymphadenopathy or hepatosplenomegaly. What is the most likely diagnosis?
Labs:
Autoimmune hemolytic anemia (AIHA)
A 5-y/o boy presents with a 2-month history of fatigue, pale skin, and poor feeding. His parents also report increased irritability after physical activity. He has a family history of thalassemia, with his father having thalassemia major. On examination, he appears pale, and mild splenomegaly is noted. His laboratory results show:
What is the appropriate treatment?
Dx: beta thalassemia
Regular blood transfusions and iron chelation therapy (for iron overload)
A 55-y/o man presents with a 3-week hx of painless neck swelling, fever, night sweats, and unintentional weight loss of 10 pounds. He also reports feeling fatigued and has had a decreased appetite. He denies any hx of recent infections or trauma. On examination, there is a large, non-tender, firm lymph node in the left cervical region. No hepatosplenomegaly is noted, and VSS. What is the dx?
Labs:
Non-Hodgkin Lymphoma
A 28-year-old woman presents with a 2-day history of swelling and pain in her left calf. She has no history of trauma but reports a family history of blood clots, with her mother having multiple episodes of deep vein thrombosis (DVT) and her brother experiencing a pulmonary embolism (PE) at a young age. On examination, the left calf is swollen, tender, and warm. Ultrasound confirms the presence of DVT. D-dimer is elevated and Protein C activity is low. What is the diagnosis?
Protein C deficiency
A 30-y/o woman presents with a 1-week hx of easy bruising and pinpoint red spots (petechiae) on her arms and legs. She denies any recent trauma, illness, or medication changes. She has no significant PMH but mentions feeling more fatigued than usual over the past few weeks.
On PE, she has multiple petechiae on her limbs, and mild ecchymosis on her legs. Her mucous membranes are not bleeding, and her vital signs are stable. There is no hepatosplenomegaly.
Lab Results: Platelet count: 12,000/μL (low), Hemoglobin, WBC, PT, aPTT normal. What is her treatment?
Dexamethasone 40 mg for 4 days
A 28-y/o man presents with a 2-mo hx of fatigue, abdominal pain, and dark-colored urine, particularly in the morning. He wakes up feeling SOB and lightheaded on several occasions. Over the past week, he has also noticed a slight increase in his bruising tendency. He denies any significant PMH or recent infections.
On PE, he appears pale, and his abdomen is slightly tender to palpation, but no hepatosplenomegaly is noted. His vital signs are stable. What is his treatment?
Laboratory Results:
Eculizumab monoclonal Ab and folic acid (supplement)
Causes of normocytic anemia
AOCD (anemia of chronic disease)/CKD
Impaired marrow function
Significant Drug Toxicities/Side Effects for Antineoplastic Agents
aD:(Adriamycin) Doxorubicin
Cardiotoxicity
B:Bleomycin
Pulmonary fibrosis
CP: Cyclophosphamide
Hemorrhagic Cystitis
V:Vincristine (Oncovin) and Vinblastine
Peripheral neuropathy
C:Cisplatin
Nephrotoxicity & Ototoxicity