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Aural pathohistology in leukemia.

Three clinical histories of leukemia are followed by their related histopathological findings, the latter through the sequence of hemorrhage, leukemic infiltration, organization to connective tissue, suppuration, formation of new bone, degeneration into cholesterol granuloma.

Adult↗

Cytology of leukemic lymphadenopathy.

OBJECTIVE: To describe the cytomorphologic features of leukemic cells in lymph node aspiration material. STUDY DESIGN: We studied lymph node fine needle aspiration (FNA) smears of 36 leukemic patients. In 23 cases the lymphadenopathy was noticed simultaneously with marrow leukemia, and in the other 13 cases the lymphadenopathy was noticed during a relapse. Special stains, such as periodic acid Schiff, Sudan Black-B, Oil Red-O and nonspecific esterase, were used in special cases. RESULTS: Thirty-three cases were diagnosed as lymphoma, 1 as extramedullary hematopoiesis and 2 as leukemic involvement. CONCLUSION: Leukemic lymphadenopathy can be misdiagnosed as lymphoma on FNA smears. The clinical findings, previous history, hematologic studies and immunocytochemical studies are essential to the differentiation of leukemic smears from lymphoma. However, in some cases the leukemic infiltration can be diagnosed with certainty, provided that the smears show the characteristic findings, such as Auer rods and neoplastic promyelocytes with azurophilic granules.

Adolescent↗

Cytology of ascitic fluid in a patient with granulocytic sarcoma (extramedullary myeloid tumor). A case report.

BACKGROUND: Granulocytic sarcoma (GS) is the rare extramedullary manifestation of acute myeloid leukemia that may precede or be concurrent with leukemic infiltration of bone marrow or herald blastic transformation of a chronic myeloproliferative disorder. It has been found in most body sites and shows no age or sex predilection, necessitating its inclusion in the differential diagnosis of undifferentiated neoplasms. CASE: A 36-year-old female presented with a three-year history of abdominal pain, jaundice and fluctuating abdominal girth. Cytology of the ascitic fluid revealed myeloid cells of eosinophilic lineage at all stages of differentiation, with many undifferentiated cells. Immunohistochemical studies on a cell block confirmed the diagnosis of granulocytic sarcoma, which excluded the differential diagnoses of Hodgkin's disease, non-Hodgkin's lymphoma and Langerhans histiocytosis. CONCLUSION: Granulocytic sarcoma may present as a serous effusion and can be diagnosed on a cytologic specimen.

Abdominal Neoplasms↗

Testicular pseudolymphoma.

Pseudolymphomas are reactive processes which by their morphology can be confused with lymphomas. They have been described in non-lymphoid organs (gastrointestinal tract, lung, breast, etc.), although cases have also been reported in the spleen. In this article we present the case of a 3-year-old child in whom the location was in the testes. It is the 4th case reported in the literature and the first in a child. The main criteria for establishing such a diagnosis are the delimitation of the lesion, the presence of mature lymphocytes intermingled with other cells which are mainly plasma cells of polyclonal type and the formation of lymphoid follicles with active germinal centers. A differential diagnosis within the testicle should be made with the lymphoma (primary or secondary), the leukemic infiltration and the seminoma with marked stromal lymphoid reaction.

Child, Preschool↗

Morphologic evidence for coronary artery spasm in eclampsia.

Vascular spasm has been considered to be an important component of the eclamptic state. If this abnormal vascular reactivity affects the coronary arteries in eclampsia, one might expect to find areas of myocardial contraction band necrosis, a lesion secondary to coronary reflow after periods of no flow. We reviewed the cardiac findings in the 34 patients with fatal eclampsia (hypertension, edema, proteinuria, and convulsions without evident cause) autopsied at The Johns Hopkins Hospital since 1899, and compared each with the next pregnant or puerperal nontoxemic autopsied patient. The eclamptic patients were 15-45 years old (average 27 years). Convulsions began antepartum in 21 patients, intrapartum in eight, and postpartum in five. The hearts weighed 200-407 g (average 312 g). One heart had rheumatic valvular disease and one had myocarditis. Histologic study of heart sections showed the presence of contraction band necrosis in 12 cases (35%). The control cases included two patients with rheumatic valvular disease, two with endocarditis, two with myocarditis, two with pericarditis, and one with leukemic infiltration. Only one control patient (3%) had contraction band necrosis (p less than 0.001). The frequent occurrence of myocardial contraction band necrosis suggests that coronary artery spasm may be common in patients who die with eclampsia.

Adolescent↗

Osteolytic lesions as a presenting sign of acute myeloid leukemia.

Osteolytic lesions rarely occur in acute myeloid leukemia (AML). We reported an atypical form of the disease, with marrow fibrosis and osteolytic lesions, in a 17-year-old patient, whose main symptom was lumbar pain. Diagnosis of AML was established by bone marrow and lymph node histological analysis. Computed tomography (CT) scan and 99mTc-MDP bone scintyscan revealed osteolytic lesions. After remission-induction, bone marrow aspirate and biopsy showed no evidence of leukemic infiltration, nevertheless bone abnormalities persisted on 99mTc-MDP bone scintyscan, suggesting residual disease. Suspect bone areas were irradiated with symptomatic improvement and 99mTc-MDP bone scintyscan showed the appearance of more condensed bone compared with the pre-radiotherapy pattern. Twelve months later he was readmitted to the hospital due to relapse of AML and died of sepsis within a few weeks. This report illustrates the usefulness of histological studies to establish diagnosis of AML in atypical cases, as well as the importance of CT scan and bone scintigraphy scan for the identification of osteolytic lesions. It also provides additional data as evidence that although osteolytic lesions indicate an adverse prognosis in AML, local irradiation results symptomatic relief.

Acute Disease↗

Endobronchial Richter's syndrome. A rare manifestation of chronic lymphocytic leukemia.

Endobronchial involvement in chronic lymphocytic leukemia is rare. This report describes a patient with chronic lymphocytic leukemia and Richter's syndrome in whom bronchial obstruction occurred due to massive peribronchial lymphadenopathy and endobronchial leukemic infiltrates. Endobronchial Richter's transformation has not been previously reported.

Bronchi↗

Inner ear hemorrhage and endolymphatic hydrops in a leukemic patient with sudden hearing loss.

Several significant histopathological findings were noted in a case of sudden hearing loss in a patient with chronic lymphocytic leukemia. The major pathological findings were leukemic hemorrhage into both perilymphatic and endolymphatic spaces in the cochlear and vestibular systems, endolymphatic hydrops in the cochlea and sacculus, and a relatively narrowed and straightened vestibular aqueduct and endolymphatic sac. Additional interesting findings include: loss of hair cells in the organ of Corti and vestibular end-organs; destruction of the stria vascularis (possibly the origin of the blood); fibrosis in the perilymphatic spaces in the cochlea and the vestibule, and in the endolymphatic space in the vestibule; and new bone formation in the perilymphatic spaces in the vestibule. The leukemic infiltrate observed in both the cochlea and the vestibule was not considered to be significant. Hemorrhage into the cochlea is thought to be the most reasonable cause of the sudden hearing loss in this case. Also discussed are fibrosis and osteogenesis as a late consequence of hemorrhage, and the coexistence of endolymphatic hydrops with an anomaly of the vestibular aqueduct and endolymphatic sac.

Aged↗

Leydig cell function in boys following treatment for testicular relapse of acute lymphoblastic leukemia.

Current practice for achieving local control of testicular relapse in males with acute lymphoblastic leukemia (ALL) includes the use of 2,400-rad testicular radiation. Although this therapy is known to cause germ cell depletion, it has been assumed that it does not alter testicular secretion of testosterone. To test this assumption, we measured gonadotropin and testosterone levels in seven boys with ALL who had been treated with radiation for clinically apparent testicular relapse. In four of seven boys, testicular relapse was bilateral with overt involvement of one testicle and microscopic involvement of the other. Three of these four boys demonstrated delayed sexual maturation, and in addition to elevated follicle-stimulating hormone (FSH) concentrations, testosterone levels were low and luteinizing hormone levels were elevated compared with controls. These data indicate that boys with overt testicular leukemia who are treated with 2,400-rad testicular radiation are at risk for Leydig cell dysfunction. However, the relative contributions of radiation, prior chemotherapy, and leukemic infiltration to this dysfunction remain to be clarified.

Adolescent↗

Phase I study of mitoxantrone plus etoposide with multidrug blockade by SDZ PSC-833 in relapsed or refractory acute myelogenous leukemia.

PURPOSE: Expression of the multidrug resistance gene (MDR1) p170 protein is frequent in leukemic blasts from patients with relapsed acute myelogenous leukemia (AML). A phase I study using the nonimmunosuppressive MDR1 blocker SDZ PSC-833 (PSC) in combination with mitoxantrone (MITO) and etoposide (VP) was performed. PATIENTS AND METHODS: Starting doses (LVL0) of MITO (3.25 mg/m2/d on days 1 and 3 to 6) and VP (210 mg/m2/d on days 1 and 3 to 5) were 40% of the maximal-tolerated dose (MTD) from a prior study. A 1.5-mg/kg loading dose of PSC was followed by a 120-hour continuous infusion of 10 mg/kg/d on days 2 to 6. Blood samples for PSC, MITO, and VP pharmacokinetics (PK) were taken on days 1 and 3, and samples for MDR1 expression were taken on day 0. RESULTS: Severe mucositis developed in all patients at LVL0; therefore, MITO and VP doses were reduced to 2.5 and 170 mg/m2 (LVL-1) for the next seven patients, and this dose proved to be MTD. All LVL0 and three LVL-1 patients had transient elevations in the serum bilirubin level to > or = 4 mg/dL. Serum creatinine level increased to greater than 2 mg/dL in one case. There were no other grade 3 or 4 nonhematologic toxicities observed. The peripheral blood was cleared of leukemia in three LVL0 and four LVL-1 patients. The marrow was cleared of leukemic cells in one LVL0 and five LVL-1 patients, and a significant reduction in marrow leukemic infiltrate was observed in eight of 10. No patient achieved complete remission (CR), and all died of progressive disease (n = 8) or infection (n = 2). MDR1 expression was detected by fluorescent-activated cell sorter (FACS) analysis in five of seven cases. An elevated MDR1 mRNA level was detected by quantitative polymerase chain reaction (Q-PCR) in six of eight cases studied. Clearing of leukemia cells from the marrow occurred in four of six MDR1-positive and one of three MDR1-negative patients. Despite the fact that LVL0 doses had to be reduced due to toxicity, coadministration of PSC did not produce a consistent effect on MITO PK; however, it did repeatedly lead to increased levels of VP in the serum. CONCLUSION: We conclude that PSC-MITO-VP is a tolerable regimen with antileukemic activity. Addition of PSC necessitated a 66% reduction in MITO and VP doses from a prior study without PSC.

Antineoplastic Combined Chemotherapy Protocols↗

Intracranial and otological presentation of acute lymphocytic leukemia.

The authors report a patient with acute lymphocytic leukemia in hematological remission who presented with both intracranial and otological manifestations of the disease. The patient presented with clinical symptoms and computed tomographic findings consistent with otomastoiditis and a contiguous brain abscess. However, both lesions were identified at operation as leukemic infiltrates. Intracranial mass lesions and otological complications associated with leukemia are reviewed.

Adolescent↗

Richter's transformation presenting as an obstructing endobronchial lesion.

A 60-year-old man with chronic lymphocytic leukemia presented to our institution with a recurring lingular pneumonia. On fiberoptic bronchoscopy, the patient was found to have an endobronchial mass obstructing the lingula and left upper lobe. Biopsy specimens of the mass demonstrated anaplastic large cell lymphoma consistent with Richter's transformation. Only one case of endobronchial Richter's transformation has been previously reported in the literature. This was described as peribronchial and endobronchial leukemic infiltrates within the bronchial mucosa. We report the first case of an obstructive endobronchial mass secondary to Richter's transformation. The endobronchial mass was treated with a Nd-YAG laser to maintain airway patency while the patient underwent chemotherapy, resulting in complete resolution of the mass within the airway.

Airway Obstruction↗

Oral manifestations of acute leukemia in children.

Seventy-seven children with acute leukemia were examined. A wide spectrum of oral findings was observed as the consequence of direct leukemic infiltration of tissue, infection resulting from bone marrow and immunosuppression, impaired healing responses, and poor oral hygiene. Guidelines for dental treatment are provided.

Adolescent↗

Trismus in a 6 year old child: a manifestation of leukemia?

Trismus is a firm closing of the jaw due to tonic spasm of the muscles of mastication from disease or the motor branch of the trigeminal nerve. Trismus may be produced by a variety of reasons such as dental abscess, trauma, following mandibular block with local anesthesia, as a result of radiation to the facial muscles, and patients after chemotherapy. A case of a referral of a six-year-old boy to a dentist from an ENT due to severe limitation in jaw opening is presented. Intraoral examination and panoramic radiograph demonstrated no signs of infection and/or other pathology. After a diagnosis of trismus was made, due to his icteric appearance, the general fatigue and loss of appetite in the last few days, palpated and sensitive lymph nodes in the submandibular and cervical regions, the child was referred for a complete blood count and sedimentation rate. The laboratory and clinical findings resulted in the diagnosis of acute lymphoblastic leukemia (ALL). Dental and oral manifestations of ALL are discussed, and the trismus may be explained by an intensive infiltration of leukemic cells into the deep portion of the contracting muscles of the face. This case emphasizes the importance of physical examination and independent judgement made by dentists, even when patients are referred to them by other members of the medical communities.

Blood Cell Count↗

Neutropenic ulceration. A distinctive clinical entity.

Neutropenic ulceration is a distinctive clinical entity observed in severely myelosuppressed patients, such as those with acute leukemia. This case report demonstrates that evidence of its initial resolution may indicate the earliest stages of bone marrow recovery before this is reflected in the peripheral circulation through the return of functioning neutrophils. The possible influences of leukemic infiltration and bacterial superinfection in its initiation and progression are discussed.

Adult↗

Diffuse pulmonary alveolar hemorrhage in acute promyelocytic leukemia.

A 60-year-old male with acute promyelocytic leukemia demonstrated bilateral diffuse air-space consolidation on chest X-ray. An autopsy on the next day revealed pure pulmonary alveolar hemorrhage without leukemic infiltration or inflammation. Disseminated intravascular coagulation was confirmed microscopically. In severe hemorrhagic diathesis and leukopenia, it is impossible to distinguish pulmonary hemorrhage from pneumonia by X-ray alone. Bronchalveolar lavage may be the only possible diagnostic approach.

Bronchoalveolar Lavage Fluid↗

Three cases of vascular problems in the human stria vascularis.

The histopathological findings of a study of temporal bones are reported. Each patient suffered from one of the following systemic vascular diseases: leukemia, hypertension, and diabetes mellitus. In each temporal bone, a differing strial pathology was observed: leukemic infiltration, dilatated strial vessels, and microaneurismata. Microvascular changes as seen in this study could make the human inner ear more vulnerable to inner ear traumata.

Adult↗

Temporal bone pathology in patients without caloric response.

Pathologies of serial sections of 9 temporal bones from 6 patients without caloric response were studied. The patients died of epipharyngeal cancer, middle ear cancer, chronic renal failure with DIC, pancreatic cancer with DIC, multiple brain metastasis of pancreatic cancer, and leukemia. Under light microscopy, 8 ears showed pathologies in the vestibular end organs and the nerves. Possible causes of the lack of caloric responses in the 8 ears were endolymphatic hemorrhage, labyrinthitis, leukemic infiltration in the labyrinth, degeneration or disappearance of the vestibular sensory cells, reduction in the number of vestibular nerve fibers, and/or brainstem lesions. The remaining one ear did not show abnormal findings in the vestibular end organs or the nerves, but the patient had brainstem lesions. Four types of temporal bone pathologies were observed according to the lesion site responsible for the lack of caloric response; i) sensory type, ii) neural type, iii) mixed type and iv) central type.

Adult↗