Bone Marrow ; pathology ; Humans ; Liver Transplantation ; adverse effects ; Lymphohistiocytosis, Hemophagocytic ; diagnosis ; Male ; Middle Aged ; Pancytopenia ; diagnosis

The cutaneous manifestations of hemophagocytic lymphohistiocytosis (HLH) are variable and nonspecific. A 42-year-old man presented with multiple annular, erythematous patches on the trunk for 3 months. Two months later, he presented with bullae along with high fever. The laboratory examination showed pancytopenia, hypertriglyceridemia, and hypofibrinogenemia. The bone marrow biopsy specimen showed an active hemophagocytosis. On the basis of these findings, a diagnosis of HLH was concluded. After five cycles of chemotherapy, his skin lesion completely resolved. Taking the results together, we suggest that annular skin lesion can be added to the list of cutaneous manifestations of HLH.
Adult ; Biopsy ; Bone Marrow ; Diagnosis ; Drug Therapy ; Fever ; Humans ; Hypertriglyceridemia ; Lymphohistiocytosis, Hemophagocytic* ; Pancytopenia ; Skin*

The cutaneous manifestations of hemophagocytic lymphohistiocytosis (HLH) are variable and nonspecific. A 42-year-old man presented with multiple annular, erythematous patches on the trunk for 3 months. Two months later, he presented with bullae along with high fever. The laboratory examination showed pancytopenia, hypertriglyceridemia, and hypofibrinogenemia. The bone marrow biopsy specimen showed an active hemophagocytosis. On the basis of these findings, a diagnosis of HLH was concluded. After five cycles of chemotherapy, his skin lesion completely resolved. Taking the results together, we suggest that annular skin lesion can be added to the list of cutaneous manifestations of HLH.
Adult ; Biopsy ; Bone Marrow ; Diagnosis ; Drug Therapy ; Fever ; Humans ; Hypertriglyceridemia ; Lymphohistiocytosis, Hemophagocytic* ; Pancytopenia ; Skin*

A 42-year-old man presented with severe pancytopenia and uncontrolled epistaxis. The diagnosis of SLE was made and the pancytopenia was found to be due to myelofibrosis. The pulse therapy with methylprednisolone and maintenance therapy with prednisolone reversed both pancytopenia and myelofibrosis. Although myelofibrosis has been described in SLE, this coexistence must be very rare since there has been only 19 cases showing this combination. We report a case of SLE with myelofibrosis which was reversed by the treatment with glucocorticoid.
Adult ; Diagnosis ; Epistaxis ; Humans ; Lupus Erythematosus, Systemic* ; Methylprednisolone ; Pancytopenia ; Prednisolone ; Primary Myelofibrosis*

Virus associated hemophagocytic syndrome, class ll histiocytoses, characterized by high fever, severe constitutional symptoms, abnormal liver function and coagulation, perigheral blood pancytopenia and histiocytic hyperplasis with prominent hemophagocytosis in bone marrow and lymph nodes has been reported and associated with active viral infection. It is non-malignant and reversible. It must be differentiated from histiocytic medullary reticulosis because of the inappopriateness of immunosuppressive of cytotozic therapy which is the therapeutic method for HMR, but is contraindicated in the treatment of VAHS. This paper describes two patients whose clinicopathology was compatible with the diagnosis of virus associated hemophagocytic syndrome.
Bone Marrow ; Diagnosis ; Fever ; Histiocytosis ; Humans ; Liver ; Lymph Nodes ; Lymphohistiocytosis, Hemophagocytic* ; Pancytopenia

Histiocytic Medullary Reticulosis(H.M.R.) is a rapidly progressing fatal disease seen most often in adults, but it has been reported in children younger than 15 years of age. H.M.R. is clinically characterized by fever, wasting generalized lymphadenopathy and hepatosplenomegaly. In the terminal stage, jaundice, purpura, anemia and pancytopenia are all present with or without skin involvement. Cardinal pathologic features are systemized proliferation of atypical, neoplastic, erythrophagocytic histiocytes and their precursors throughout the lymphoreticular tissues. Four cases of H.M.R. occurring in pediatric age group, i.e., 5 years, 6 years, 12 years and 14 years of age, respectively, are presented with discussion of the clinico-pathologic characteristics and management. Cases 1,2 and 3 died within 6 months after the onset of illness. Case 1 of our series was particularly younger than in previously reported cases. This was the case that the diagnosis was confirmed by postmortem examination. Analysis of previously reported 25 cases occurring in ages younger than 20 years of age was done.
Adult ; Anemia ; Autopsy ; Child* ; Diagnosis ; Fever ; Histiocytes ; Humans ; Jaundice ; Lymphatic Diseases ; Pancytopenia ; Purpura ; Skin

Gastric cancer is a major cause of cancer-related mortality. At the time of diagnosis, majority of the patients usually have unresectable or metastatic disease. The most common sites of metastases are the liver and the peritoneum, but in the advanced stages, there may be metastases to any region of the body. Bone marrow is an important metastatic site for solid tumors, and the prognosis in such cases is poor. In gastric cancer cases, bone marrow metastasis is usually observed in younger patients and in those with poorly differentiated tumors. Prognosis is worsened owing to the poor histomorphology as well as the occurrence of pancytopenia. The effect of standard chemotherapy is unknown, as survival is limited to a few weeks. This report aimed to evaluate 5 gastric cancer patients with bone marrow metastases to emphasize the importance of this condition.
Bone Marrow* ; Diagnosis ; Drug Therapy ; Humans ; Liver ; Mortality ; Neoplasm Metastasis* ; Pancytopenia ; Peritoneum ; Prognosis ; Stomach Neoplasms

Histiocytic medullary reticulosis was first described as a clinical and pathological entity by Scott and Robb-Smith in 1939. The clinical pictures include progressive pancytopenia, fever, wasting, lymphadenopathy, hepatosplenomegaly, jaundice and purpura. The outstanding morphological features of the disease is proliferation of histiocytes throughout the reticuloendothelial system with frequent leucoerythrophagocytosis. In spite of uniform clinical and fistologcal pictures verified by additional case reports, the diagnosis has usually not been made until postmortem examination. Most patients die within six months because the disease runs a rapidly fatal course. Since the orginal report, approximately 90 cases have been reported in the literatures. In this ciuntry, Ko and others reported several cases of HMR in adults, and there has been only one report in childhood. Recently, we had the opportunity to observe a 9 year old girl at this hospital whose clinical course and antemortem pathological features were consistent with criteria for the disease. In spite of the remission after treatment with various anticancer drugs, patient expired six months after diagnosis.
Adult ; Autopsy ; Child ; Diagnosis ; Female ; Fever ; Histiocytes ; Humans ; Jaundice ; Lymphatic Diseases ; Mononuclear Phagocyte System ; Pancytopenia ; Purpura

OBJECTIVETo study the clinical features and etiological spectrum of pancytopenia in children.

METHODSThe clinical data of 174 children with pancytopenia between September 2003 and January 2010 were retrospectively reviewed.

RESULTSPale face was the most common clinical manifestation (147 cases, 84.5%), followed by bleeding (87 cases, 50.0%) and fever (41 cases, 23.6%). Mild to moderate anemia, severe thrombocytopenia and mild leucopenia were common in complete blood count. Of the 174 children, pancytopenia was attributed to hematopoietic system diseases in 155 cases (89.1%) and non-hematopoietic system diseases (virus infections, systemic lupus erythematosus, hypersplenism and neuroblastoma) in 6 cases (3.4%). Aplastic anemia (91 cases, 52.3%) was the most common cause of pancytopenia, followed by myelodysplastic syndrome (37 cases, 21.3%), acute leukemia and other hematological tumours (11 cases, 6.3%) and hemophagocytic syndrome (6 cases, 3.4%). The cause of pancytopenia was not identified in 13 cases (7.5%).

CONCLUSIONSAnemia, bleeding and fever are the main clinical manifestations of pancytopenia in children. Pancytopenia is mostly caused by aplastic anemia in children. Myelodysplastic syndrome, hematological tumours and hemophagocytic syndrome are also the common causes.

Adolescent ; Child ; Child, Preschool ; Female ; Humans ; Infant ; Male ; Pancytopenia ; blood ; diagnosis ; etiology

Trichosporon beigelii belongs to the family of Cryptococcaceae. T. beigelii is a rare causative agent of invasive pneumonia in immunocompromised patients. T. beigelii pneumonia is characterized by persistent fever unresponsive to broad-spectrum antibiotic the- rapy, coughing with bloody sputum, and rapidly progressive dyspnea. Its diagnosis should be based on clinical suspicion and confirmed by culture and histologic examination of biopsy tissue. We describe an ALL patient with T. beigelii pneumonia developed during severe pancytopenic period after salvage chemotherapy.
Biopsy ; Cough ; Diagnosis ; Drug Therapy* ; Dyspnea ; Fever ; Humans ; Immunocompromised Host ; Pancytopenia ; Pneumonia* ; Sputum ; Trichosporon*