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It accounts for the majority of cases of acute leukemia in adults B cheap 100 mg clomid. Patients are more likely to have hepatosplenomegaly and lym- phadenopathy at presentation D order clomid 25 mg line. Maintenance chemotherapy generally lasts 1 to 3 years E. The Philadelphia chromosome–positive (Ph+) variant is more resistant to standard treatment Key Concept/Objective: To know the differences between AML and ALL in adults AML accounts for about 80% of acute leukemias in adults and is most likely to present with hemorrhage or infection. Standard induction therapy with cytarabine and daunoru- bicin (7 + 3 regimen) is followed by consolidation chemotherapy but generally no long- term maintenance regimen. ALL typically presents with constitutional symptoms (fatigue, weight loss, night sweats), and organomegaly and lymphadenopathy are more likely to be present on exam. Because CNS involvement occurs in 5% of patients with ALL, CNS pro- phylaxis is a standard part of treatment, as is maintenance chemotherapy. Ph+ ALL is less responsive to standard chemotherapy regimens. A 47-year-old man presents with gum bleeding, rectal bleeding, and fatigue. He is found to have dis- seminated intravascular coagulation (DIC). Which of the following conditions would best fit with this clinical presentation? Ph+ ALL Key Concept/Objective: To recognize acute promyelocytic leukemia as a distinct disease with a significant associated complication DIC is frequently found at presentation or soon after induction of chemotherapy in patients with acute promyelocytic leukemia (FAB M3). Hemorrhage secondary to DIC is responsible for a high pretreatment or early-treatment mortality. Acute promyelocytic leukemia is unique in its response to all- 42 BOARD REVIEW trans-retinoic acid, which is used alone or in combination with more standard regimens for induction. A 55-year-old man presents to your clinic with complaints of generalized fatigue, weight loss, and abdominal discomfort with early satiety. On physical examination, the patient is afebrile and appears thin. His abdominal examination is notable for massive splenomegaly. No adenopathy is identified, and the liver is of normal size. A complete blood count (CBC) reveals a neutrophilic leukocytosis, and you suspect chronic myelogenous leukemia (CML). Which of the following statements regarding CML is false? CML is a myeloproliferative disorder (MPD) and represents a clonal disorder of the pluripotential hematopoietic stem cell B. The CBC often reveals thrombocytosis, neutrophilic leukocytosis, and basophilia C. The presence of the Philadelphia chromosome (Ph) is characteristic of CML and is a poor prognostic sign D. The three main phases of CML are the chronic phase, the accelerated phase, and the blast phase Key Concept/Objective: To understand the pathogenesis and clinical course of CML MPDs represent clonal disorders of the pluripotential hematopoietic stem cell and include CML, polycythemia vera, essential thrombocythemia, myeloid metaplasia, and idiopathic myelofibrosis. CML accounts for 15% of all cases of leukemia in adults. Males are affected more often than females, and the median age at presentation is 45 to 55 years. CML is caused by the transforming capability of the protein products resulting from the Ph translocation t(9;22). Up to 95% of patients with CML express Ph, which results from a reciprocal translocation between the long arms of chromosomes 9 and 22. Patients with CML who do not have Ph translocation have a significantly worse prognosis than do patients who test positive for the bcr-abl gene. CML is characterized by expansion of myeloid progenitor cells at various stages of their maturation, by the premature release of these cells into the circulation, and by their tendency to home to extramedullary sites. Symptoms at presentation reflect the increase in mass and turnover of the leukemic cells.

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These complications can range from increased pain—observed in 25% of patients—to infection order clomid 25mg online, sensory loss generic clomid 50mg on-line, and neurologi- cal and vascular injury. Further complications include increased anesthesia time and operative blood loss as well as prolonged recovery time. In addition to the procedural complications, the supply of bone available for harvest is limited. These complications, along with the risk of donor site morbidity and the limited supply of material available for harvest, have pushed the search for alternative materials. ALLOGRAFT BONE Allograft bone is the substitute most often used for autograft bone. It is typically available fresh, frozen, or freeze-dried. This material undergoes extensive processing and donor screening in an effort to reduce the risk of disease transmission. This processing decreases but does not eliminate the risk of disease transmission. In addition, the processing decreases the mechanical and biological properties of the bone, while adding to the cost of the material. In summary, although a viable alternative to autograft bone, allograft bone suffers from concerns of possible disease transmission, recipient rejection, increased cost, and limited availability. SUBSTITUE BONE GRAFT MATERIALS The problems associated with autograft and allograft materials described above, not the least of which is the increasing demand for a limited supply, have acted to fuel the development of substitute materials. Substitute materials have primarily been developed as a replacement for graft materials and currently are used in approximately 10% of the bone graft procedures per- formed worldwide. Calcium salt ceramics and glasses, such as calcium phosphates and hydroxyapatites, are widely used synthetics. Demineralized bone and collagen-based materials have also been used as substitute bone graft materials. Calcium phosphate materials such as tricalcium phosphate (TCP) and hydroxyapatite (HA) are among the most common synthetics. These materials have been successfully applied in orthopedic and dental applications for decades. Calcium salt materials have compositions similar to that of native bone and provide osteoconductive surfaces on which new bone can form. Although somewhat similar in composition, the materials elicit different biological responses. The TCP material is readily resorbed, whereas the HA is a more or less permanent material, taking several years to be removed and replaced by native bone. TCP typically is more porous than HA, helping it to be resorbed more rapidly. Most TCP and HA materials are able to provide a highly osteoconductive structure; however, the materials lack or have limited osteoinductive properties. In addition, the materials are often brittle, making them unable to support physio- logical loads without additional internal or external support. Commercially available products include Interpore Internationals ProOsteonTM(Irvine, CA), which is a coral-derived HA material. The material was the first synthetic approved by the Food and Drug Administration (FDA). A Polymer Bone Graft Extender 161 As with most materials of this type, it is highly osteoconductive, with limited osteoinductive properties and limited mechanical strength. Norian has developed an injectable form of calcium phosphate, the Norian SRS (skeletal repair system). The calcium and phosphate powder are mixed with solution to form a paste that can be injected or packed into a defect site. External or internal reinforcement is usually required to provide adequate support. Demineralized bone matrix (DMB) is a highly processed allograft material. Treatment of bone with mild acid removes the mineral component of bone, while leaving growth factors and proteins. These growth factors and proteins are mixed with a substrate, such as glycerol, to form a workable material. Materials of this type typically have limited osteoconductive proper- ties, but good osteoinductive properties.

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She has a history of rheumatoid arthritis and has been on hydroxy- chloroquine order 100 mg clomid with visa, 400 mg/day discount clomid 25 mg fast delivery, and prednisone, 5 mg/day, for several years. On examination, the eye is very red, with a violaceous hue to the sclera. Gentle finger pressure over the eyelid onto the globe is painful. Which of the following should be the next step in the care of this patient? Call for an ophthalmology appointment Key Concept/Objective: To be able to recognize serious eye disease in rheumatoid arthritis Patients with rheumatoid arthritis may have a variety of eye problems, including dry eye, episcleritis, and scleritis. Dry eye is rarely serious and is treated with eyedrops and lubri- cants. Episcleritis is inflammation of superficial vessels and is generally not a threat to vision; scleritis is caused by inflammation of the deeper vessels and can lead to loss of vision. Differentiation of the two is based on the more violaceous hue of the sclera in scle- ritis—caused by inflammation around the sclera vessels—and pain on pressure over the closed lid onto the globe, which is not seen in episcleritis. Confirmation can be done by 8 BOARD REVIEW slit-lamp examination. Scleritis comes in different forms, and treatment decisions are best made in conjunction with an ophthalmologist. The most serious form of scleritis can lead to scleromalacia perforans, which usually leads to blindness in the affected eye. Hydroxychloroquine can cause eye disease, notably retinopathy, but there are no superfi- cial manifestations. A 55-year-old man comes to your clinic with a persistent cough. He had a viral syndrome 3 weeks ago that has cleared, but he continues to have a nonproductive cough. Past medical history is significant for seropositive rheumatoid arthritis and a 25-pack-year history of smoking. Current medications include leflunomide, 10 mg/day, and prednisone, 5 mg/day. Physical examination is significant for mild ulnar deviation of the fingers and fibular deviation of the toes, but little active synovitis. Rheumatoid nodules are present over the extensor surface of both forearms near the elbows. Chest x-ray reveals a 2 cm × 2 cm pulmonary nodule in the right upper lobe but is otherwise normal. Which of the following should be the next step in the care of this patient? Perform a CT scan to evaluate the lesion further E. Schedule a transbronchial biopsy Key Concept/Objective: To understand the evaluation of pulmonary nodules in patients with rheumatoid arthritis Patients with rheumatoid arthritis, particularly men with subcutaneous nodules who are smokers, are prone to developing rheumatoid nodules in the lung. They can be of various sizes, may be single or multiple, and tend to be peripheral in location. Unfortunately, those patients who are at risk for rheumatoid lung nodules are also at risk for lung cancer, and pulmonary nodules in patients with rheumatoid arthritis should be considered potentially malignant. A CT scan of the chest is the most reasonable first step to evaluate location and the presence of adenopathy. In most cases, a biopsy will be necessary for histologic evaluation. A 35-year-old woman comes to clinic for follow-up of rheumatoid arthritis and to evaluate a new rash on the lower extremities. She was diagnosed with rheumatoid arthritis 5 years ago on the basis of joint pain and a positive rheumatoid factor, but the rheumatoid factor has been intermittently positive since then. Physical examina- tion is significant for the lack of synovitis in the small joints of the hands and feet and the presence of palpable purpura on both lower extremities.

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