Heme/Onc Flashcards
Acute GVHD (within 100 days post BMT) Clincal presentation
-Skin: painful maculopapular rash that can progress to toxic epidermal necrolysis,
-GI: abdominal pain, diarrhea, and elevated liver enzymes (eg, bilirubin, alkaline phosphatase).
This syndrome typically occurs within a median time of 19 days after HCT.
Acute GVHD: Risk factors
-human leukocyte antigen mismatch or unrelated donor
-gender disparity (eg, female donor to male recipient)
-lack of prophylactic GVHD regimen (eg, methotrexate, cyclosporine).
Acute GVHD treatment
Steroids with cyclosporine and Methotrexate
Chronic GVHD onset
Chronic GVHD resembles an autoimmune vascular disease and can occur in up to 50% of HCT patients at any time after transplant (median of 201 days post HCT).
Chronic GVHD Risk factors
older age of donor/recipient,
prior acute GVHD,
seropositive cytomegalovirus in either donor or recipient.
Chronic GVHD presentation
Skin: skin rash resembling lichen planus or scleroderma with areas of hypo- or hyperpigmentation.
GI: increased liver function tests (eg, bilirubin, alkaline phosphatase), dry oral mucosa with ulcerations, esophageal webs/strictures
Opth: dry eyes with possible cataracts
lung: bronchiolitis obliterans,
MSK: fasciitis or joint strictures.
Chronic GVHD diagnosis
Diagnosis is usually clinical but may require skin or gastrointestinal biopsy
Chronic GVHD treatment
Localized therapy (eg, topical steroids for skin involvement, cyclosporine eye drops) is preferred for limited chronic GVHD.
Extensive chronic GVHD requires systemic steroids with cyclosporine or tacrolimus.
Haiy cell leukemia
Rare, mature B-cell lymphoproliferative disorder that typically causes splenomegaly and pancytopenia.
peripheral blood smear shows tumor cells that are 1-2 times the size of normal lymphocytes with “hairy” projections.
Confirmation is made with peripheral blood flow cytometry and trephine bone marrow biopsy with immunohistochemical stains.
Type 1 heparin-induced thrombocytopenia
Non-immune-mediated thrombocytopenia due to an immediate (within 48 hours) reaction to heparin therapy.
Platelet counts rarely drop to <100,000/mm³ and usually recover spontaneously regardless of heparin continuation or discontinuation
Acute intermittent porphyria
Patients presenting with recurrent acute abdominal pain combined with dark urine and hyponatremia should be evaluated for acute intermittent porphyria. The diagnostic test is an elevated urinary porphobilinogen (PBG) during an acute attack. Treatment involves glucose loading and intravenous hemin
Tamoxifen preOp
It exerts estrogen- mimetic effects on the coagulation system and is associated with increased rates of venous thromboembolism (VTE).
Risk of VTE is particularly high during the first 2 years of therapy and in those who require prolonged immobilization due to surgery (or travel).
As a result, patients who take tamoxifen for primary prophylaxis should discontinue the medication 2-4 weeks prior to any surgery associated with a moderate or high risk of VTE (eg, hip replacement).
Those on tamoxifen for cancer treatment should discuss perioperative cessation with their oncologist.
Procedures associated with a low risk of VTE usually do not require tamoxifen cessation
TLS management
Patients at high risk for tumor lysis syndrome from chemotherapy should be treated with aggressive hydration (to maintain a urine output of >80-100 mL/hr), rasburicase, and careful clinical and biochemical monitoring.
CLL definition
Accounts for nearly 30% of adult leukemias in the United States and has a median age of 70 at diagnosis.
Patients typically present with “B symptoms” (eg, recurrent fever, night sweats, weight loss), painless lymphadenopathy, hepatosplenomegaly, anemia, and thrombocytopenia.
Diagnosis is confirmed by peripheral smear showing ≥5000/mm³ mature-appearing small B lymphocytes and flow cytometry showing monoclonal B-cell characteristics (eg, CD19, CD20, CD23).
CLL indication of treatment
CLL is usually an indolent disease with a median survival of >10 years in asymptomatic patients with early-stage disease (Rai stage 0-1, Binet stage A). As a result, asymptomatic patients do not need treatment and can be observed for possible disease progression
Indication:
• Systemic symptoms (eg, fever, weight loss, fatigue, night sweats)
• Massive/progressive lymphadenopathy or hepatosplenomegaly
• Progressive marrow failure (eg, anemia, thrombocytopenia)
• Autoimmune hemolytic anemia or thrombocytopenia unresponsive to steroids
• Rapidly progressive lymphocytosis (doubling time <6 months)
• Repeated infections
Post Radiation screening
In patients who received chest radiation, annual screening mammograms are recommended starting 8-10 years after radiation therapy. Annual TSH testing is also recommended in these patients.
Complex regional pain syndrome
Complex regional pain syndrome commonly presents after an injury with throbbing pain, paresthesias, skin temperature changes, and local edema. Diagnosis is suggested clinically by symptoms and abrupt symptom relief with a local anesthetic block.
Dabigatran pre-op
The ongoing physiologic effects of dabigatran, a direct thrombin inhibitor used as an oral anticoagulant, can be assessed using thrombin time:
A)Normal thrombin time: The patient is not anticoagulated surgery can proceed without additional interventions.
B)Elevated thrombin time: The patient is currently anticoagulated administration of the dabigatran antidote idarucizumab should be considered in patients with life- threatening bleeding and those who require urgent/emergency surgery.
Patients on dabigatran undergoing elective procedures are generally advised to discontinue the medication 48 hours prior to the operation (as the half-life is approximately 12 hours). These individuals usually do not require a thrombin time
Post polycythemia Vera myelofibrosis
Diagnostic criteria
Major
• Previous diagnosis of PV
• Presence of increased bone marrow fibrosis
Minor
• Progressive anemia, loss of requirement for phlebotomy, or cytoreductive therapy
• Leukoerythroblastic blood picture
• Increasing splenomegaly
• Development of constitutional symptoms (fever, weight loss, and night sweats)
Treatment for post-PV myelofibrosis is similar to that for primary myelofibrosis. The only definitive treatment is allogenic hematopoietic stem-cell transplantation. Patients with severe splenomegaly and debilitating constitutional symptoms can be treated with JAK2 inhibitors (eg, ruxolitinib).
Priapism in sickle cell management
Priapism is a serious complication associated with sickle cell disease and defined as an erection lasting >2-4 hours. Blood aspiration from the corpus cavernosum, followed by saline irrigation and use of adrenergic agonists (eg, epinephrine), is first-line therapy. If priapism still persists, surgical shunting may be required.
Transfusion associated GVHD
Introduction
a rare condition that occurs when donor lymphocytes from transfused blood attack host tissue. Nearly all cases arise in patients who are immunocompromised
Unlike GVHD associated with hematopoietic stem cell transplantation, ta-GVHD causes bone marrow destruction leading to progressive pancytopenia, which is the major underlying cause of morbidity and death.
Transfusion associated GVHD
Presentation and treatment
Manifestations typically occur 4-30 days after transfusion and include fever and an erythematous/maculopapular rash that often becomes generalized; additional features include anorexia, (right upper-quadrant) abdominal pain, hepatomegaly, liver function abnormalities, and profuse water diarrhea (>7-8 L/day).
Treatment is typically ineffective, and most cases (80%-90%) are fatal. Therefore, prevention is paramount. Those at high risk for the condition (eg, immunocompromised state) should receive irradiated blood products to inactivate donor lymphocytes prior to transfusion. Patients with HIV do not appear to be at risk for this condition and can receive nonirradiated blood (possibly because HIV alters donor lymphocyte survival)
Borderline Vit B12
Vitamin B12 deficiency is common in patients with advanced age and may present with neuropsychiatric findings and no hematologic findings. Laboratory evaluation that reveals a borderline vitamin B12 level requires further testing with methylmalonic acid (MMA) level. A high level of MMA confirms vitamin B1, deficiency.
Multiple myeloma with negative SPEP
Multiple myeloma (MM) is usually associated with a monoclonal spike on serum protein electrophoresis (SPEP). However, this test often is unable to identify patients who produce excessive light chains rather than full immunoglobulin. Therefore, patients with classic symptoms of MM who have negative SPEP should undergo urine electrophoresis with immunofixation or serum free light-chain assay, which can identify monoclonal light chains.
Patients with negative SPEP, UPEP, and serum FLC analysis who have classic symptoms of MM and bone marrow biopsy evidence of ≥10% clonal plasma cells have “nonsecretory” myeloma, which is seen in ~3% of cases.
Sickle cell and strokes
Patients with sickle cell disease are at high risk for ischemic or hemorrhagic stroke.
Exchange transfusion is recommended for acute stroke;
scheduled simple transfusions are recommended to prevent recurrent strokes. Done every 4-6 weeks with goal Hb S levels of <30% and total Hb levels of 9-12.5 mg/dL.
This strategy reduces the risk of stroke recurrence from 65% to <20% and is superior to medical management with hydroxyurea.
Post orthopedic surgery VTE prophylaxis
For patients undergoing orthopedic surgery without increased bleeding risk, postoperative dual venous thromboembolism prophylaxis with intermittent pneumatic compression and low-molecular-weight heparin is recommended during hospitalization; low-molecular-weight heparin should be continued for up to 35 days.
Myeloid reconstitution syndrome
Previously febrile neutropenic patients experiencing myeloid recovery may develop recurrent fever and the appearance or worsening of an inflammatory focus, a condition known as myeloid reconstitution syndrome. Although affected patients should be closely monitored for infection, no immediate treatment change is typically required.
Polycythemia
Polycythemia is defined as a hemoglobin level >16 g/dL in women and >16.5 g/dL in men and is typically categorized into primary and secondary causes. Primary polycythemia is erythropoietin (EPO)-independent and is usually associated with low EPO levels; polycythemia vera is the most common cause and is usually diagnosed by JAK2 mutation testing and bone marrow biopsy. Secondary polycythemia is usually EPO-dependent and marked by high EPO levels; hypoxia is the most common cause and is typically diagnosed by oxygen saturation testing at rest, after exercise, and during sleep.
Renal cell carcinoma
Signs and symptoms
• Can be asymptomatic
• Hematuria (only with invasion of collecting system)
• Abdominal mass, abdominal pain, weight loss
• Scrotal varicocele
Renal cell carcinoma
Para neoplastic syndrome
• Anemia (microcytic or normocytic)
• Erythrocytosis (increased erythropoietin production)
• Nonmetastatic liver function abnormalities
• Fever & weight loss
• Hypercalcemia (due to bone metastases,↑ PTHrP, or ↑ prostaglandins)
• AA amyloidosis
Erythromelalgia
Definition and presentation
a condition that causes burning pain and erythema (sometimes pallor and/or cyanosis) in the extremities.
It is likely due to intermittent blood vessel obstruction.
Episodes are usually triggered by heat, exertion, stress, insomnia, or pressure.
Symptoms can occur in the symmetric extremities (lower > upper), ears, or face. Patients typically avoid wearing socks or shoes since the generated heat can precipitate attacks. Elevating the legs can also help relieve symptoms
Erythromelalgia
Causes and treatment
Erythromelalgia can be either primary or secondary, and is usually due to an underlying myeloproliferative disorder with platelet counts > 400,000 cells/µl.
Diagnosis is usually clinical as there are no specific diagnostic tests.
Treatment is directed at the underlying disorder (if secondary), but aspirin may reduce symptoms in both primary and secondary erythromelalgia.
Aspirin is also helpful in reducing vascular events in patients with essential thrombocythemia.
In addition, patients are instructed to elevate their extremities, cool the affected area, and avoid heat or any other triggers.
Prostate cancer radiation complications
Sexual dysfunction is the most common long-term adverse effect of external beam radiation for prostate cancer. The risk depends on the radiation technique and radiation field. The risk increases further if androgen deprivation therapy is added to the radiation treatment.
Small cell lung carcinoma management
Patients with SCLC are categorized based on radiograph and physical examination findings as follows:
Limited stage - tumor limited to the ipsilateral hemithorax and regional lymph nodes (although subclinical metastases are usually present)
Extensive stage - involvement of contralateral lymph nodes, malignant pericardial/pleural effusion, or distant metastases (as in this patient with liver and contralateral lung lesions)
Platinum-based chemotherapy (eg, etoposide plus cisplatin) is highly effective for inducing remission in SCLC. Patients with limited-stage SCLC are treated with curative intent and usually receive concurrent chemotherapy and thoracic radiation; 5-year survival is ~20%. Extensive-stage SCLC is generally treated with 6 cycles of chemotherapy alone; those who respond to chemotherapy often receive subsequent chest and/or prophylactic cranial irradiation (Choice E). Five-year survival is ~5%.
targeted drug therapy is not currently part of first-line treatment for SCLC, molecular profiling is not typically performed.
Delayed onset HIT
Delayed-onset, heparin-induced thrombocytopenia is an immune-mediated condition that usually arises 5-19 days after heparin cessation. Most patients present in the outpatient setting with thrombocytopenia and arterial or venous thrombus. HIT antibody testing is required for confirmation, but empiric treatment with a nonheparin anticoagulation (eg, direct thrombin inhibitor) should be initiated with urgency to prevent life-threatening complications.
