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lymphohistiocytic hemophagocytosis

Prasan Kumar Panda, Ramjas Prajapati, Arvind Kumar, Manisha Jana, Pradeep Immanuel, Pranay Tanwar, Naveet Wig
A young adult man with 4-years history of lepromatous leprosy (received irregularly multidrug therapy) presented with two and half years history of symptoms suggestive of chronic erythema nodosum leprosum (ENL), initially responded to steroids and thalidomide, but later on failed. During the last 2-months, he developed fever, vomiting, and subsequently altered sensorium. On evaluation, he had hepatosplenomegaly, hyponatremia, hyperferritinemia, hemophagocytosis in bone marrow aspiration, lobular panniculitis in skin biopsy, and multiple parenchymal nodules in chest imaging...
August 2017: Intractable & Rare Diseases Research
Jose Luis Ramírez-Bellver, Joaquin Lopez, Elena Macias, Laura Fuertes, Irene Andres, Victoria Alegria, Ignacio Gimeno, Alejandra Perez, Yosmar Perez, Luis Requena
Fixed drug eruption (FDE) consists of recurrent dusky-red to brownish macules or patches at the same sites after the readministration of the causative drug. It usually presents as a solitary lesion, but generalized eruptions have been described. The most frequently implied drugs are antibiotics, anticonvulsants, and analgesics. Only 2 cases due to metformin have been reported. Histopathologic features of FDE include vacuolar degeneration of the basal layer, necrotic keratinocytes, and superficial and deep perivascular lymphocytic infiltrate...
June 2017: American Journal of Dermatopathology
M Teresa Fernández-Figueras, M Teresa Martín-Urdà, Adrià Plana, Octavi Servitje, Rosa M Penin, Gustavo Tapia, José L Mate, Aurelio Ariza
AIMS: Many types of intravascular lymphohistiocytic proliferation have been described recently; this was previously an unnoticed or misinterpreted phenomenon. Intralymphatic lymphohistiocytic aggregates are relatively common, and include benign, malignant and indeterminate conditions. In contrast, all non-endothelial proliferations in the lumina of blood vessels have been interpreted so far as malignant. Herein, we present three cases of histiocytic proliferations in the lumen of blood vessels associated with intracytoplasmic granulocyte debris (haemophagocytosis), a previously undescribed entity...
December 2016: Histopathology
G Mellon, B Henry, O Aoun, D Boutolleau, A Laparra, J Mayaux, M Sanson, E Caumes
INTRODUCTION: Adenoviral infection is a classic cause of lymphohistiocytic hemophagocytosis (LH) in bone marrow transplantation but is rare outside this setting. CASE REPORT: A 31-year-old female, with a history of treated mesencephalic astrocytoma, was hospitalized for fever, pancytopenia, elevated liver enzymes, hyperferritinemia and hypertriglyceridemia. Adenovirus viral load in blood was 7.3×10(9) copies/mL. Bone marrow aspirate examination confirmed LH. The patient recovered without specific LH or adenovirus-directed treatment...
May 2016: Journal of Clinical Virology: the Official Publication of the Pan American Society for Clinical Virology
Umit Yavuz Malkan, Gursel Gunes, Tuncay Aslan, Sezgin Etgul, Seda Aydin, Yahya Buyukasik
Hemophagocytic syndrome (HPS) is described by an increase in macrophages accountable for extensive phagocytosis of hematopoietic cells. Secondary HPS arises commonly in the presence of infections, neoplasia, autoimmune disorders and immune disorders. Here, we reported a patient with common variable immune deficiency (CVID) and Hodgkin's lymphoma (HL) who later developed EBV linked hemophagocytic lymphohistiocytosis. 42 year old men underwent check-up because of back pain in July 2012. He had known CVID disease...
2015: International Journal of Clinical and Experimental Medicine
Lucy Lu, Shuoyan Ning, Zain Kassam, Richard Hunt, Marco Puglia
A 70-year-old woman with celiac disease presented with weight loss and diarrhea unresponsive to gluten-free diet (GFD) and prednisone. Diagnosis of type 2 refractory celiac disease (RCD) was made by small intestinal biopsies showing severe villous blunting and intraepithelial lymphocytosis. She was diagnosed with hemophagocytic lymphohistiocytic syndrome (HLH) after developing fever, pancytopenia, hypofibrinogenemia, elevated ferritin, and demonstration of hemophagocytosis on her bone marrow biopsy. An expert pathologist on lymphoma reviewed her biopsies and revised the final diagnosis to type 1 enteropathy-associated T-cell lymphoma (EATL) based on large T-cells infiltrating the lamina propria...
April 2014: ACG Case Reports Journal
Dennis J Chute, James Rawley, John Cox, Robert J Bready
The hemophagocytic lymphohistiocytosis (HLH) syndrome is a hyperimmune disorder characterized by lymphohistiocytic infiltrations, elevated cytokine levels in the blood, macrophage activation, and hemophagocytosis, frequently presenting with a febrile septic picture. This unusual disease is more common in infancy and childhood than adulthood. It is classified as primary or familial when a genetic defect is identified and secondary or acquired when triggered by certain infections, autoimmune disorders, or malignancies...
July 2013: Journal of Forensic Sciences
Brian Belyea, Ashley Hinson, Cassandra Moran, Eugene Hwang, Jessica Heath, Raymond Barfield
Secondary hemophagocytic lymphohistiocytosis (sHLH) is a reactive, proliferative disorder of the immune system resulting in lymphohistiocytic proliferation, hemophagocytosis, and cytokine dysregulation. The most common infectious trigger in sHLH is Epstein-Barr virus (EBV-HLH). Current treatment protocols for EBV-HLH have a cure rate of approximately 75%; however, there are significant toxicities associated with these therapies. We present two patients with EBV-HLH who experienced spontaneous resolution of their disease prior to the initiation of therapy, suggesting there may be a subgroup of patients with EBV-HLH who do well with conservative management and can avoid potentially toxic therapies...
October 2010: Pediatric Blood & Cancer
Jey-Hsin Chen, Mark D Fleming, Geraldine S Pinkus, Jack L Pinkus, Kim E Nichols, Jun Q Mo, Antonio R Perez-Atayde
Familial hemophagocytic lymphohistiocytosis is a rare, rapidly progressive disorder characterized by an activation of the immune system resulting in a systemic proliferation of lymphocytes and histiocytes. The disease is genetically heterogeneous and maps to at least 4 loci including the gene encoding perforin, a protein critical for the cytotoxic and regulatory functions of T lymphocytes and natural killer (NK) cells. Hepatic dysfunction often occurs early in the clinical course, but the pathology of the liver is not well characterized...
June 2010: American Journal of Surgical Pathology
Mohamed E Salama, David M Parham, Sherrie L Perkins, David W Bahler, Dale A Ellison
Most Epstein-Barr virus (EBV)-related infections in infants and children are asymptomatic or self-limited mild viral illnesses, but rare cases of a rapidly fatal disorder have been described. Failure of the cellular response to control EBV-related lymphoid proliferation leads to severe disease with multiple complications, including a fatal outcome or development of an EBV-driven, clonal lymphoid neoplasm. In this report we characterize 3 cases of fatal, nontransplant, or immunodeficiency-related EBV infection in very young children with immunophenotypic and molecular evidence of B/natural killer (NK)-T cell clonal expansion...
November 2008: Pediatric and Developmental Pathology
Klaus Kapelari, Martin Fruehwirth, Andreas Heitger, Alfred Königsrainer, Raimund Margreiter, Burkhard Simma, Felix Albert Offner
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare, fatal disorder of early infancy. We report two siblings with FHL whose symptoms were dominated by hepatic failure. Both presented with sudden-onset fever and hepatosplenomegaly with progressive abnormalities of clinical biochemistry indices of liver function. One died of hepatorenal failure. The other underwent liver transplantation. Autopsy and explant liver displayed portal and periportal infiltrates of T lymphocytes and histiocytes; an activation of the hepatic mononuclear phagocytic system with focal hemophagocytosis; and almost complete loss of interlobular bile ducts...
June 2005: Virchows Archiv: An International Journal of Pathology
E Tugrul Saribeyoglu, S Anak, L Agaoglu, O Boral, A Unuvar, O Devecioglu
Since the first description of infection-associated hemophagocytosis (IAHS), the list of precipitating infectious agents causing hemophagocytic syndrome has grown. A lymphohistiocytic proliferation with hemophagocytosis may develop as a result of macrophage activation, viral or bacterial infection, parasitic infestations, or malignancy. The authors report on a 3-year-old boy with Langerhans cell histiocytosis (LCH), who developed IAHS during malaria infection. Hemophagocytic syndromes may complicate the course of LCH and cause diagnostics problems...
April 2004: Pediatric Hematology and Oncology
Nandita Kakkar, R K Vasishta, A K Banerjee, R K Marwaha, B R Thapa
We describe four classical cases of familial hemophagocytic lymphohistiocytosis (FHL), a macrophage-related, autosomal recessive fatal disorder. Parental consanguinity was present in three cases. All patients presented with fever, neurological involvement of varying degrees, hepatosplenomegaly, cytopenias, deranged liver function tests, and coagulogram, hypofibrinogenemia (three cases), and hyperlipidemia (one case). An antemortem diagnosis could not be made, although it was suspected in one case. Necropsy (done in three cases and postmortem liver biopsy in one case) revealed classical features of FHL...
May 2003: Pediatric Pathology & Molecular Medicine
Elizabeth M Kfoury Baz, Abdel Razzak A Mikati, Nadim A Kanj
Hemophagocytic lymphohistiocytosis (HLH) is characterized by fever, cytopenia, splenomegaly, and lymphohistiocytic proliferation with hemophagocytosis. Sporadic, familial, and reactive HLH varieties exist. The latter, also termed the reactive hemophagocytic syndrome (RHS), has been associated with a variety of infectious and noninfectious etiologies. Activation of monocytes in RHS is due to stimulation by high levels of activating cytokines. RHS has not been associated previously with thrombotic thrombocytopenic purpura (TTP)...
April 2002: Therapeutic Apheresis
R Zermani, K M'Rad, F el Mezni, O Khayat, S Ben Becher, S Ben Jilani
No abstract text is available yet for this article.
March 1998: La Tunisie Médicale
C Boissy, P Velin, J F Michiels, J Diebold, P Hofman
We report 2 cases of familial hemophagocytic lymphohistiocytosis in two children, hétérozygous twins, born from consanguine parents. This disease is characterised by disseminated lymphohistiocytic infiltrates with hemophagocytosis, that most commonly involves bone marrow, spleen, lymph nodes, liver and central nervous system. Differential diagnosis is difficult with infection-induced hemophagocytic syndromes. The only distinguishing feature in pathology is the expression of CD21, CD30 and CD35 antigens by histiocytes...
1997: Archives D'anatomie et de Cytologie Pathologiques
J F Silverman, H K Singh, V V Joshi, C T Holbrook, A R Chauvenet, L S Harris, K R Geisinger
Familial hemophagocytic syndrome (FHS) is a rare, fatal disorder of childhood demonstrating failure to thrive, fever, hepatosplenomegaly (HSM), recurrent infections, pancytopenia, and histologically, the infiltration of reticuloendothelial organs by benign-appearing histiocytes demonstrating hemophagocytosis. We report two fatal cases of FHS including a 3 year-old female who underwent fine-needle aspiration (FNA) biopsy of the liver in the initial workup of the disease (case 1) and an 8 month-old boy with ascites and HSM having peritoneal fluid cytology submitted as the first specimen for morphologic examination (case 2)...
August 1993: Diagnostic Cytopathology
A M Marty, J S Dumler, G Imes, H P Brusman, L L Smrkovski, D M Frisman
Human ehrlichiosis is a tick-borne zoonosis caused by the newly described human hematotropic rickettsiae, Ehrlichia chaffeensis. The pathology and pathogenesis of human ehrlichiosis have not been adequately studied. Even with immunoperoxidase, the only previously known method to detect these organisms in tissue, ehrlichae are difficult or impossible to identify. This led many investigators to speculate that the pathogenesis of ehrlichiosis was not caused directly by the organism but could be caused by host-mediated injury...
August 1995: Human Pathology
A Labbe, P Dechelotte, F Demeocq, G Lesec, J Gaulme
Two brothers presented with hemophagocytic reticulosis with neonatal onset. Early clinical and biological symptoms included pallor, hepatosplenomegaly and anemia, thrombocytopenia. Evolution was lethal in both cases, at 5 months and 13 days of age, respectively. Diagnosis was confirmed in both by the pathologic findings: diffuse lymphohistiocytic cellular proliferation with hemophagocytosis and atrophy of the lymphoid tissue. The diagnostic difficulties of this disease in the neonatal period are emphasized.
October 1982: Archives Françaises de Pédiatrie
D Soffer, E Okon, N Rosen, B Stark, C Hershko
Presented are the pathologic findings in familial hemophagocytic lymphohistiocytosis (FHLH), based on observation of 11 cases from four affected families. The outstanding morphologic feature was a multiorgan involvement with lymphohistiocytic cellular infiltrates. The organs most frequently affected were the bone marrow and lymph nodes and, less frequently, the liver and the brain. The histiocytes in the infiltrates were usually bland and displayed prominent hemophagocytosis. The differential diagnosis of FHLH and the difficulty in distinguishing it from malignant histiocytosis is discussed...
December 1, 1984: Cancer
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