Philadelphia-positive T-cell acute lymphoblastic leukemia with polymyositis, migratory polyarthritis and hypercalcemia following a chronic myeloid leukemia

Transformation of chronic myeloid leukemia (CML) often results in acute myeloblastic or, less frequently, in precursor B-cell acute lymphoblastic leukemia (ALL). T-cell blast crisis is rare. Hypercalcemia has also been described as a rare complication of CML, but this usually occurs as a terminal event. Here we report a case of a 35-year-old woman who developed a CD4(+)/CD8(+) T-cell ALL 2 years after the diagnosis of a typical Ph(+) CML. Polymyositis and polyarthritis preceded by 4 months, and symptomatic hypercalcemia occurred just before blastic transformation, probably representing paraneoplastic manifestations of the disease.     

Sarcoidosis with hypercalcemia, urolithiasis, renal insufficiency, and heart failure]

A 65-year-old man was admitted to our hospital because of thirstyness and left lower abdominal pain. On admission, he was found to have urolithiasis, renal insufficiency (BUN: 73 mg/dl, Crt: 4.4 mg/dl), and hypercalcemia (13.2 mg/dl). Chest X-ray films and computed tomograms showed enlargement and calcification of the hilar lymph nodes, and thickened interlobar fissures in both lungs. Levels of angiotensin converting enzyme (30.2 IU/l) and 1.25 (OH)2VitD3 (66.4 pg/ml) were elevated. Histologic examination of the specimen obtained from transbronchial lung biopsy showed non-caseous epithelioid cell granulomas. Because the level of parathyroid hormone was normal and no malignancies were detected, a diagnosis of sarcoidosis was made. Treatment with extracorporeal shock wave lithotripsy, transurethral lithotomy, saline infusion, and prednisolone (30 mg/day) alleviated the urolithiasis, renal insufficiency, and hypercalcemia. After discharge, the patient was followed up and given prednisolone therapy. About 1 month after the prednisolone dose had been tapered to 15 mg/day, the patient experienced dyspnea and facial and pedal edema. Because congestive heart failure was diagnosed, he was re-admitted to our hospital for a second time. Although he was then placed on intensive therapy, he died of ventricular tachycardia associated with sarcoidosis of the heart.     

Sarcoidosis initially manifesting as symptomatic hypercalcemia with the absence of organic involvement

A 53-year-old man was admitted to Osaka City University Hospital on July 21, 1998, for investigation of symptomatic hypercalcemia. Laboratory data on admission revealed that serum Ca had increased to around 12.6 mg/dl and there was a significant increase in urinary Ca excretion. The serum phosphate level remained normal. Although the serum PTH level was below the detection limit, serum 1,25-dihydroxyvitamin D (1,25(OH)2D) was increased. Diagnosis of sarcoidosis was supported by a negative tuberculin test and by the elevated levels of serum angiotensin-converting enzyme (ACE), lysozyme activity, and CD4/CD8 ratio in bronchoalveolar lavage specimen; there was however no imaging evidence of sarcoidosis such as bilateral hilar lymphnode enlargement on chest X-ray, high resolution CT or 67Ga citrate scintigraphy. Biopsy specimens from the cervical lymphnode revealed no epitheloid cell granulomas or giant cells. Administration of prednisolone achieved a decrease in serum ACE and 1,25(OH)2D levels, followed by restoration of serum Ca and urinary Ca excretion to the normal range, and finally by an increase of serum PTH to the normal level. These observations suggested that the hypercalcemia could be explained by extrarenal production of 1,25(OH)2D. We report here on this rare case of sarcoidosis with initial symptoms of symptomatic hypercalcemia resulting from extrarenal production of 1,25(OH)2D.     

Multiple-crystal acute polyarthritis

A patient with acute polyarthritis due to crystal-positive simultaneous gout and pseudogout affecting different joints is described. The case emphasises the importance of aspirating more than one involved joint and carefully searching for crystals in patients with acute arthritis involving 2 or more joints, particular those in whom the diagnosis of multiple crystalline joint disease is considered.     

Sarcoidosis associated with lupus pernio and acute pulmonary cavitation]

Lupus pernio, an uncommon skin condition due to sarcoidosis in Japan, is a chronically persistent, violaceous skin lesion primarily involving the nose, cheek, ears, and fingers. It has often been associated with chronic fibrotic non-resolving pulmonary sarcoidosis. We reported a case of sarcoidosis associated with lupus pernio and primary pulmonary cavitation as a rare manifestation in the lung. A 44-year-old man visited our hospital in January 1986 because of a 3-year history of swollen and violaceous cheeks (lupus pernio) and an 8-year history of erythema in both upper and lower limbs. The biopsy specimen obtained from the skin lesion revealed epithelioid cell granulomas without any evidence of Mycobacterium or fungal growth. Serum ACE (45.4 U/ml) was elevated. Chest X-ray films and computed tomographic (CT) scans showed bilateral hilar lymphadenopathy, nodules, multiple small opacities, and reticulo-linear opacities. Gallium scintigraphy demonstrated abnormal uptake in the mediastinal and hilar lymph nodes and lung fields. The patient had not received corticosteroids. Chest X-ray films and CT scans in July 1989 showed increased opacities and cavitation. Because pyogenic bacteria and acid-fast bacilli were not detected by repeated sputum examinations or bronchial washing, we concluded that the cavitary lesions were manifestations of primary pulmonary cavitation due to sarcoidosis. Prednisolone (40 mg daily) was prescribed and the dosage was gradually tapered. The lung lesions gradually resolved and have not recurred.     

Sarcoidosis manifesting as acute appendicitis

PURPOSE: This report presents a patient who developed signs and symptoms of acute appendicitis because of sarcoid involvement of the appendix. METHODS: This is a retrospective case review with review of the English language literature. RESULTS: The patient underwent appendectomy for suspected acute appendicitis. Histologically the appendix had no signs of acute inflammation but did have noncaseating granulomas. The patient's abdominal pain resolved following appendectomy, and she has had no further similar pain in the two years since the operation. CONCLUSION: Patients with sarcoidosis may develop signs and symptoms of acute appendicitis without inflammation. Operative exploration should not be delayed in equivocal cases of right lower quadrant abdominal pain in patients with sarcoidosis.     

Meningococcemia presenting as acute polyarthritis.

Four patientw with acute polyarthritis due to meningcoccemia are described. One had skin manifestations suggesting vasculitis, another had transient nodular lesions, but there were none of these features in the other two. Synovial fluid cultures were negative and cells counts did not suggest septic arthritis. The diagnosis was not suspected until positive blood cultures were reported in three cases and meningitis developed in the fourth. In spite of diagnostic delay due to slow growth in cultures (six to 11 days) there was prompt response to antibiotic treatment. Meningococcemia should be suspected in patients presenting with fever and polyarthritis, even in the absence of skin lesions.     

Sarcoidosis presenting with acute pleurisy, hemoptysis, pruritus and eosinophilia

Sarcoidosis carries a low prevalence in Israel, and acute pleural involvement in sarcoidosis is uncommon throughout the world. We report a case of a young Israeli male of Yemenite origin who presented with atypical manifestations of sarcoidosis: pleuritic pain, hemoptysis, pruritus and alcohol-induced pain. The differential diagnosis from Hodgkin's disease was involved. Various aspects of diagnosis are discussed.     

Hypocalcemia and hypercalcemia in patients with rhabdomyolysis with and without acute renal failure

Patients with rhabdomyolysis (RBD) and acute renal failure (ARF) are hypocalcemic during the oliguric phase of ARF and over 30% develop hypercalcemia during the diuretic phase. The present study examined the factors underlying these derangements in calcium metabolism in 15 patients: 7 with RBD and ARF, 4 with RBD only, and 4 with ARF only. All patients had hypocalcemia on admission and the hypocalcemia was more pronounced in those with RBD and ARF. All patients with RBD independent of the presence or absence of ARF had calcium deposition in soft tissues as documented by technetium-99 scan. In 4 patients with RBD and ARF, hypercalcemia developed during the diuretic phase at a time when Serum PTH levels were undetectable. Only patients with RBD and ARF had a significant increase in serum levels of 25-hydroxyvitamin D and 1,25-dihydroxyvitamin D [1,25(OH)2D] during the diuretic phase and both the increments in and the levels of 1,25(OH)2D were significantly greater in those who were hypercalcemic. The data indicate that 1) hypocalcemia occurs in RBD independent of ARF and is most likely related to calcium deposition in injured tissues, and 2) elevation in serum levels of 1,25(OH)2D plays an important role in the genesis of hypercalcemia during the diuretic phase of patients with RBD and ARF. Our observations suggest that extrarenal production of 1,25(OH)2D may occur in these patients, and/or that the renal production of 1,25(OH)2D may not be so tightly controlled as it is in normal subjects.     

Case report: metolazone-associated hypercalcemia and acute pancreatitis

Metolazone-induced acute pancreatitis and hypercalcemia are described in a 58-year-old woman with severe congestive cardiac failure. Her symptoms and laboratory abnormalities rapidly resolved upon discontinuation of metolazone. Both clinical and laboratory findings make other etiologies for the patient's pancreatitis extremely unlikely. The pathophysiology of thiazide-related hypercalcemia and pancreatitis is reviewed. To our knowledge, neither hypercalcemia nor the combination of acute pancreatitis with hypercalcemia has been reported previously in association with metolazone therapy, and the association of pancreatitis and metolazone has been noted previously only once.     

Sarcoidosis with primary acute cavitation exacerbated by bronchial asthma]

A 22-year-old woman complaining of blurred vision visited our hospital in March 1995 and was given a diagnosis of uveitis. Chest X-ray and computed tomographic (CT) films demonstrated bilateral hilar lymphadenopathy (BHL), diffuse granular shadows in both lung fields, and a cavity with a thin, smooth wall in the right upper lung field. Because histopathologic findings from transbronchial lung biopsy specimens of the lung and cavity tissues disclosed sarcoid granuloma, the diagnosis was sarcoidosis with primary cavitation, which is very rare for this disease. Although BHL disappeared without medication, in November the patient experienced dyspnea due to attacks of bronchial asthma that had been in remission for a long period. Treatment with inhalation of becromethazone propionate markedly alleviated her symptoms. In May 1998, follow-up chest CT films demonstrated that the cavity had disappeared. This case suggested that sarcoidosis leads to a deterioration of asthma control, a conclusion supported by previous reports.