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Two cases of myelodysplastic syndrome with extramedullary polyclonal plasma cell proliferation and autoantibody production: possible role of soluble Fas antigen for production of excessive self-reactive B cells.

Two cases of myelodysplastic syndrome (MDS) with extramedullary polyclonal plasma cell proliferation and autoantibody production are reported. These cases, which showed leukemic change of refractory anemia with excess of blast (RAEB), developed lymph node swelling and muscle abscess; both were infiltrated mainly with plasma cells, without preceding infection. The proliferation of plasma cells was polyclonal and was proven by negative rearrangement of immunoglobulin heavy chain gene or polyclonal staining of immunoglobulin light chains. These patients showed polyclonal gammopathy and autoantibo

Sampling variability on percutaneous liver biopsy.

Sampling variability of liver biopsy was determined in three consecutive biopsy specimens obtained from each of 118 patients immediately prior to autopsy. No sampling variability was found for fatty liver, alcoholic hepatitis, nonspecific hepatitis, fulminant hepatitis, leukemic infiltrate, and venous congestion. Cirrhosis was diagnosed in 80% of cases at the first biopsy but in all cases after three biopsies. Chronic aggressive and chronic persistent hepatitis were diagnosed correctly in two of three cases each at the first biopsy, and in all cases after three biopsies. Metastatic carcinoma w

Intrathoracic manifestations of hematologic malignancies: a narrative review.

BACKGROUND AND OBJECTIVE: Although the global incidence of hematologic malignancies is decreasing, the risk of developing these cancers is increasing due to increased lifespan and modern treatments including targeted therapy, chemotherapy, and radiation. Intrathoracic manifestations of hematologic malignancies may be the presenting sign leading to diagnosis or result from various treatment toxicity. The most common respiratory manifestation of hematologic malignancies is infectious, but up to half of pulmonary findings are from non-infectious etiologies. We aim to summarize the current literat

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… Leukemic infiltration can also rarely present as nodules and be seen in a peribronchovascular, centrilobular, or random distribution. … Radiographic infiltrates are reported in less than 7% of patients with leukemia but may be underdiagnosed, as histologic evidence of leukemic infiltration at autopsy ranges from 38% to 60% ( 35 ). …

Detection of Minimal Residual Disease in Adult Acute Lymphoblastic Leukemia by Analysis of Gene Rearrangements and Correlation with Early Relapses.

While more than 75% of the adult patients with acute lymphoblastic leukemia (ALL) achieve a complete remission after treatment with intensive chemotherapy, about 40% of them relapse within five years. These relapses are probably due to residual leukemic cells. Gene rearrangements are used as markers of clonality and thereby monoclonal leukemic lymphoid cells can be detected with high sensitivity. In this study, we have applied the analysis of gene rearrangements to detect minimal residual disease in patients considered to be in complete remission. Serial bone marrow samples were studied in 35

Usefulness of bronchoalveolar lavage and flow cytometry in patients with hematological malignancies and respiratory failure.

BACKGROUND AND OBJECTIVES: Strategies to improve the efficiency of bronchoalveolar lavage (BAL) are needed. We conducted a study to establish the diagnostic value of BAL in patients with hematological malignancies and pulmonary infiltrates. PATIENTS AND METHODS: The correlation of cytologic and flow cytometric study of BAL with the microbiological findings and the clinical evolution was determined. RESULTS: Seventy BAL were performed and flow cytometric study was analyzed in 23 of them. Fifty-three patients did not present any adverse event attributable to BAL. Anti-infectious therapy was mo

Blueberry muffin baby: a pictoral differential diagnosis.

The term blueberry muffin baby was initially coined by pediatricians to describe cutaneous manifestations observed in newborns infected with rubella during the American epidemic of the 1960s. These children had generalized hemorrhagic purpuric eruptions that on histopathology showed dermal erythropoiesis. Since then, congenital infections comprising the TORCH syndrome (toxoplasmosis, other, rubella, cytomegalovirus, herpes) and hematologic dyscrasias have classically been associated with blueberry muffin-like lesion.

A case of hypopyon uveitis nonresponsive to steroid therapy and a review of anterior segment masquerade syndromes in childhood.

We report a case of hypopyon uveitis referred as inflammatory disease nonresponsive to corticosteroids. A detailed history revealed that the patient had been treated previously for acute lymphoblastic leukemia (ALL). Anterior chamber tap was consistent with recurrence of ALL. A review of pseudouveitis in the pediatric population is also presented.

The infant skull: a vault of information.

The art of interpreting skull radiographs is slowly being lost as trainees in radiology see fewer plain radiographs and depend more heavily on computed tomography and magnetic resonance imaging. Nevertheless, skull radiographs still provide significant information that is helpful in finding pathologic conditions and appreciating their extents. Abnormalities in the skull may be reflected as variations in the density, size, and shape of the skull, as well as skull defects. Skeletal dysplasias may manifest as a generalized decrease in calvarial density (hypophosphatasia, osteogenesis imperfecta),

Pictorial review: benign and malignant enlargement of the pterygo-masseteric muscle complex.

Seven cases of unilateral enlargement of the pterygoid and/or masseter muscles due to haemangioma (1), benign masseteric hypertrophy (2), rhabdomyosarcoma (2), leukaemic infiltration (1) and non-Hodgkin's lymphoma (1) are presented. The differential diagnosis of pterygo-masseteric muscle enlargement is outlined and the usefulness of computed tomography (CT) discussed.

Milky spots as the implantation site for malignant cells in peritoneal dissemination in mice.

We examined the site-specific implantation of cancer cells in peritoneal tissues after an i.p. inoculation of 10(5) P388 leukemia cells. Twenty-four h after the inoculation, the number of viable cancer cells infiltrating into specific tissue sites of the peritoneum was estimated by an i.p. transfer method. A descending order of tissue implantation with cancer cells was established as omentum > gonadal fat > mesenterium > posterior abdominal wall > stomach, liver, intestine, anterior abdominal wall, and lung. A significant correlation was established between the logarithm of the number of infil

Cutaneous Hematolymphoid and Histiocytic Proliferations in Children.

This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Because lymphoblastic leukemias and lymphomas are more frequent in the pediatric setting, cutaneous leukemic infiltrates are relatively common in this age-group. Similarly, hi

Detection of c-kit point mutation Asp-816 --> Val in microdissected pooled single mast cells and leukemic cells in a patient with systemic mastocytosis and concomitant chronic myelomonocytic leukemia.

The c-kit mutation Asp-816-->Val is detectable not only in neoplastic mast cells (MCs) in patients with systemic mastocytosis (SM) but also in most associated hematologic non-MC lineage disease (AHNMD). In order to prove a monoclonal disease evolution we investigated DNA of pooled microdissected single cells for the presence of the mutation in a patient with SM and concomitant chronic myelomonocytic leukemia (CMML). LightCycler melting curve analysis and direct sequencing of nested polymerase chain reaction (PCR) products revealed the c-kit mutation in tryptase-positive MC and in leukemic CD15

Leukemia cutis presenting as a scrotal ulcer.

A patient with acute nonlymphocytic leukemia developed a painful scrotal ulcer thought initially to be caused by infection. The lesion failed to heal with oral antibiotic therapy and local wound care. Histopathologic examination of a biopsy specimen revealed an infiltrate of leukemic cells. This cutaneous lesion heralded the relapse of acute myelogenous leukemia. A review of the literature indicates that acute nonlymphocytic leukemia rarely presents as an ulcer or on the genitalia, thus emphasizing the uniqueness of this case regarding morphology, and site of presentation. To our knowledge, th

Changes in the compositions of the null cell compartment with murine autoimmunity.

The subpopulations that comprise the null cell compartment were examined sequentially in various strains of autoimmune-prone mice. Different patterns emerged that were consistent within strains but differed from strain to strain. Abnormalities appear earlier in life in short-lived mice, such as male BXSB and MRL/l mice, than in relatively long-lived strains, such as female BXSB and NZB mice. The accumulation of T cells in MRL/l mice was accompanied by null cell changes that contrasted with those that developed in AKR/J mice after their spleens were infiltrated with leukemic T cells. It would s

Febrile neutrophilic dermatosis associated with acute leukemia.

Febrile neutrophilic dermatosis (Sweet's Syndrome) is an uncommon syndrome characterized clinically by fever, neutrophilia, and erythematous, non-ulcerating papules or plaques on the face, neck, upper thorax, and extremities. A case associated with acute myelogenous leukemia is reported and nine other reported cases associated with acute non-lymphocytic leukemia are reviewed. The appearance and location of the lesions on the face, neck, upper trunk and extremities, a female predominance, an associated fever, marked dermal infiltrates of mature neutrophils, and rapid clearing of lesions with pa

Flow cytometric analysis of cerebrospinal fluid improves detection of leukaemic blasts in infants with acute lymphoblastic leukaemia.

Infants with acute lymphoblastic leukaemia (ALL) have a high frequency of central nervous system (CNS) involvement. Flow cytometric analysis of cerebrospinal fluid (CSF) was recently demonstrated to be a sensitive method for detecting CNS involvement in childhood ALL. In the present study, CSF from 14 infants was collected at routine lumbar punctures and analysed by multicolour flow cytometry. At initial diagnosis, leukaemic blasts were detected in CSF by flow cytometry in 11 patients (78·6%) compared to seven patients (50%) by cytospin. Larger studies are needed to determine if CSF flow cytom

Daclizumab-induced adverse events in multiple organ systems in multiple sclerosis.

OBJECTIVE: To report 3 patients with multiple sclerosis (MS) who presented with daclizumab-related adverse events (AEs) in multiple organ systems. METHODS: A retrospective chart review was performed of patients with MS who had clinical and histopathologic findings suggestive of daclizumab-induced AEs between 2004 and 2010 at the Johns Hopkins MS Clinic. This study met criteria for exemption from review from the institutional review board. RESULTS: Of 20 total patients with MS who had been treated with daclizumab, 3 patients with clinical and histopathologic findings suggestive of daclizumab-

Non-thrombotic superior sagittal sinus occlusion with intracranial hypertension following metastatic Burkitt's lymphoma.

Intracranial metastasis is a known complication of Burkitt's lymphoma, however, superior sagittal sinus invasion by dural metastasis from Burkitt's lymphoma is rare. We report a young adult, known case of Burkitt's lymphoma, who presented with features of raised intracranial pressure secondary to dural sinus invasion from metastasis. Prompt radiotherapy to these lesions can bring about recanalization of the sinus with elevation of symptoms.

Relapse of T-cell all after stem cell transplant presenting as hypertrophic cardiomyopathy: the value of non-invasive diagnostic imaging in detecting cardiac leukemia.

We describe a 14-year-old female with acute lymphoblastic leukemia (ALL) with a mediastinal mass at diagnosis who developed hypertrophic cardiomyopathy (HC) after stem cell transplantation (SCT). During refractory relapse after SCT using bone marrow from her HLA-matched sibling, she underwent whole thorax irradiation because of pleural effusion and a recurrent mediastinal mass. After a second SCT using peripheral blood from the same donor, she developed HC suspected to be related to tacrolimus. However, isolated cardiac relapse was finally diagnosed by several non-invasive imaging techniques.