Sinus histiocytosis with massive lymphadenopathy (SHML) is a benign proliferating histiocytic disorder, predominantly of lymph nodes with extra-nodal involvement in some cases. It is a self-limiting disease and has a good prognosis; however some patients need steroid therapy. Therein lays a need to differentiate it from other lympho proliferative disorders of poorer prognosis. Clinically, it is...

Recently, we evaluated a 4-year-old boy with fever, abdominal pain and multiple reddish blue subcutaneous nodules of the low back and the extremities, especially of the lower legs for 10 days. Laboratory studies showed leukocytosis, thrombocytopenia and liver dysfunction. The bone marrow aspiration excluded a proliferative disease of the haematopoietic or lymphatic system. Serological tests exc...

Multicentric reticulohistiocytosis (MRH) is a rare histiocytic proliferative disorder of uncertain etiology, characterized by mucocutaneous papulonodular lesions and progressive, symmetric erosive arthritis. MRH can coexist with various autoimmune disorders, tuberculosis, and malignancy. It usually occurs in the elderly and is very rare in children. This is probably the first case in which dise...

PATIENT Female, 72 FINAL DIAGNOSIS: Histiocytic endometritis Symptoms: Vaginal discharge • fever • weakness MEDICATION - Clinical Procedure: Endometrial and cervical biopsy Specialty: Pathology • Gynaecology. OBJECTIVE Rare disease. BACKGROUND Histiocytic or xanthogranulomatous endometritis, characterized by disappearance of endometrial mucosa and its replacement by sheets of lipid contai...

Background. Rosai-Dorfman disease (RDD) is a benign histiocytic proliferative disorder of unknown etiology. This rare condition commonly causes massive cervical lymphadenopathy. Intracranial RDD without any nodal involvement is extremely rare. Case Report. A young Bangladeshi male complained of bilateral complete blindness with left sided deafness for about three years. There was no lymphadenop...

Pulmonary histiocytic sarcoma is a rare, but highly malignant disease. Its low incidence imposes significant difficulties on physicians confronted with affected patients. The reported patient is a 24-year old male with histiocytic sarcoma of the lung. Left lower lobectomy was performed. Histologically, tumor cells had no features of carcinoma. Several entities were raised as differentials: larg...

Langerhans cell histiocytosis (LCH) is a rare histiocytic neoplasm. The clinical presentation and the disease extension are variable. LCH may involve single site, multiple sites within system, or systems. Gastrointestinal tract involvement most often observed in children with multisystem disease. Adult patients gastrointestinal extremely rare, only few cases have been reported. We present 45-ye...

Eosinophilic granuloma was formerly mistaken for “eosinophilic myeloma” (Finzi 1929) or for “osteomyelitis with eosinophilic reaction” (Schairer 1938). It was described as a new distinct entity by Otani and Ehrlich (1940) under the denomination solitary granuloma of bone and by Jaffe and Lichtenstein (1944) as eosinophilic granuloma ofbone. Farber(l94l) and Green and Farber (1942) showed that e...

We present an interesting case of a 37-year old man with acute renal failure following a febrile illness. Laboratory results showed features of macrophage activation syndrome (MAS) with anemia, thrombocytopenia, hypofibrinogenemia and elevated ferritin levels. Renal biopsy was then done to determine the cause of renal failure and showed unique glomerular findings with massive histiocytic infilt...

Histiocytic sarcoma is an uncommon malignancy in both humans and veterinary species. Research exploring the pathogenesis of this disease is scarce; thus, diagnostic and therapeutic options for patients are limited. Recent publications have suggested a role for the NLR, NLRX1, in acting as a tumor suppressor. Based on these prior findings, we hypothesized that NLRX1 would function to inhibit tum...