Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Chronic Neutrophilic Leukemia (CNL) is a rare myeloproliferative disorder characterized by a persistent increase of mature peripheral neutrophils, myeloid hyperplasia in bone marrow, hepatosplenomegaly, elevated neutrophil alkaline phosphatase (NAP) and absence of Philadelphia chromosome, with no evidence of infection or malignancy sufficient to mimic a leukemoid reaction. CNL has been associated with multiple myelomas in many reported cases, but transition of Polycythemia Vera (PV) to CNL is very rare. An 81-year-old female patient, who had undergone intermittent phlebotomy following the diagnosis of PV 8 years previously, was admitted to our hospital due to lower back pain. A physical examination showed a splenomegaly 2 cm below the costal margin, with tenderness of the thoracic and lumbar spine area. A peripheral blood examination showed a WBC count of 91,800/microL (neutrophil 88%) with a rare immature form, hemoglobin of 9.1 g/dL and a platelet count of 1,661,000/microL. Her NAP score was 58. The bone marrow examination showed 95% cellularity, with an M:E ratio of 10:1, increased megakaryocytes with normal morphology and the absence of myelofibrosis. Chromosomal studies showed no Philadelphia chromosome. A radiological examination showed compression fractures of the vertebrae and spinal cord compression. No underlying disease causing a leukemoid reaction was detected. With iron replacement, the hemoglobin level failed to increase over 12 g/dL. Therefore, it was concluded to be a transition of PV to CNL. After administration of hydroxyurea and vertebroplasty, the symptom improved and the WBC count was sustained below 40,000/microL.
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PMID:A case of transition of polycythemia vera to chronic neutrophilic leukemia. 1568 21

Leukaemoid reaction is a rare, growth factor-driven, paraneoplastic manifestation of hepatocellular carcinoma. It may masquerade as the neutrophilic chronic myeloid leukaemia or as chronic neutrophilic leukaemia. A 52-year-old male presented with hepatosplenomegaly and severe leucocytosis. He had progressive leucocytosis, neutrophil alkaline phosphatase score elevated, liver function tests altered. FNAC from the mass in the liver revealed features of moderately differentiated hepatocellular carcinoma. The patient deteriorated within two weeks and died thereafter.
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PMID:Hepatocellular carcinoma presenting as neutrophilic leukaemoid reaction--a rare entity. 1823 11

We diagnosed an 86-year-old woman with chronic neutrophilic leukemia (CNL) because she showed sustained leukocytosis dominated by mature neutrophils, hepatosplenomegaly, high neutrophilic alkaline phosphatase score, absence of the Ph chromosome, low serum level of granulocyte colony-stimulating factor (G-CSF) and granulocyte-macrophage colony-stimulating factor (GM-CSF), and no evidence of leukemoid reaction. We found that the extent of stimulation of her neutrophil functions by G-CSF and GM-CSF was greatly reduced compared to healthy donars neutrophils. We showed that CNL neutrophils have intact expression of granulocyte colony-stimulating factor receptor (G-CSFR) and granulocyte-macrophage colony-stimulating factor receptor (GM-CSFR). This suggests that failure of enhancement by G-CSF and GM-CSF in CNL neutrophil functions might be due to disturbances in the intracellular domains of G-CSFR and GM-CSFR, regardless of external cytokine stimulation. However, the patient's neutrophils did not show any mutations in the G-CSFR and GM-CSFR intracellular critical regions. We also showed that stat3 and mitogen-activated protein kinase activation by G-CSF or GM-CSF in the patient's neutrophils were significantly lower than those in healthy donor neutrophils. These results suggest that deficiency of CNL neutrophil function might be due to insufficiency of some inflammatory cytokine-specific signaling. Hence, we are the first to show that CNL neutrophils have partially insufficiency in some cytokine-specific signaling. Further studies are needed to elucidate the signal transduction pathways relating to functional defects in CNL neutrophils.
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PMID:Neutrophil function and cytokine-specific signaling in chronic neutrophilic leukemia. 1824 Dec 14

Disseminated tuberculosis can present in various ways including prolonged fever / pyrexia of unknown origin, hepatosplenomegaly, lymphadenopathy, meningitis and rarely extreme forms of hematological abnormalities such as pancytopenia and leukemoid reaction. We hereby report a case who presented with short history of fever, associated with vomiting and altered sensorium. He also had evidence of meningitis on neuroimaging with equivocal CSF finding. During his stay, he showed a spectrum of interesting hematological findings, including severe pancytopenia on peripheral smear, hemophagocytosis, epithelioid cell granuloma with Langhans' giant cells and focal necrosis consistent with tuberculosis on bone marrow examination. He showed an excellent clinical as well as hematological response to four drug antitubercular treatment (RHZE). The report highlights the significance of hematological picture in final confirmation of tuberculosis which may otherwise be passed off as nutritional or other unrelated causes.
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PMID:Disseminated tuberculosis: interesting hematological observations. 2104 80

Though commonly encountered, extrapulmonary tuberculosis (TB) can sometimes present with variable clinical picture intricating the diagnosis (Avasthi et al., 2010). The nonspecific symptoms include pyrexia of unknown origin, hepatosplenomegaly, lymphadenopathy, meningitis, and, rarely, variety of hematological abnormalities, namely, anemia, pancytopenia, and leukemoid reaction (Avasthi et al., 2010). When it presents with bone marrow (BM) involvement, prognosis is usually poor (Avasthi et al., 2010, Qasim et al., 2003, and Singh et al., 2001). We, hereby, report a case of extra-pulmonary TB with a six-month history of fever associated with dizziness, fatigability, and cough. During the hospital stay, the patient showed a spectrum of interesting hematological findings, including severe pancytopenia on peripheral smear, necrotizing caseating granulomas consistent with TB on bone marrow examination. The patient showed a good clinical as well as hematological response to antituberculosis treatment. This paper highlights the significance of a hematological picture in the final confirmation of TB, which may otherwise be passed off as nutritional or other unrelated causes.
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PMID:Unusual manifestation of extrapulmonary tuberculosis. 2371 Jan 89

Leukemoid reaction, defined as a total leukocyte count of >50,000/mm, is most commonly related to antenatal administration of steroids, infections, and transient myeloproliferative disorder of Down syndrome in newborns. Atypical presentations of viral infections can be a diagnostic challenge in the newborn period. Cytomegalovirus (CMV) infection causes a multisystem disease, and symptomatic infants generally present with intrauterine growth restriction, hepatosplenomegaly, cholestasis, rash, thrombocytopenia, and microcephaly. We present a case of a preterm infant with severe myeloid leukemoid reaction (leukocyte count >100,000/mm) at birth who was diagnosed with congenital CMV infection on the basis of CMV polymerase chain reaction results after the appearance of cholestasis, blueberry muffin rash, and hepatosplenomegaly.
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PMID:Severe leukemoid reaction in a preterm infant with congenital cytomegalovirus infection. 2407 52

Background. Myeloid sarcoma is an extramedullary neoplasm of immature myeloid cells. Our study reports a presentation of myeloid sarcoma which presented with severe leukemoid reaction as a secondary malignancy in a patient who was treated for acute lymphoblastic leukemia previously. The case emphasizes the difficulties in diagnosis of patients who do not have concomitant leukemia. Case Presentation. A 6-year-old girl who was treated for acute lymphoblastic leukemia previously presented with fatigue, paleness, and hepatosplenomegaly. Peripheral blood smear and bone marrow aspirate examination did not demonstrate any blasts in spite of severe leukemoid reaction with a white cell count 158000/mm(3). FDG/PET CT revealed slight uptake in cervical and supraclavicular lymph nodes. Excisional lymph node biopsy was performed from these lymph nodes and it showed myeloid sarcoma. Conclusion. Myeloid sarcoma can develop as a secondary malignancy in children who are treated for acute lymphoblastic leukemia. It can be associated with severe leukemoid reaction and diagnosis may be difficult if there is not concomitant leukemia. PET/CT is helpful in such cases.
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PMID:Myeloid sarcoma presenting with leukemoid reaction in a child treated for acute lymphoblastic leukemia. 2527 45

A two-month-old male infant presented with a clinical picture suggestive of fever without focus. Treatment was initiated with broad spectrum intravenous antibiotics. The infant subsequently developed septic shock, hepatosplenomegaly and tachypnoea with oxygen dependency. There was laboratory evidence of anemia, thrombocytopenia and a leukemoid reaction. Infection induced hemophagocytic lymphohistiocytosis with a leukemoid reaction was suspected because of fever, splenomegaly and bicytopenia in conjunction with elevated serum triglycerides and ferritin. Empiric therapy with doxycycline caused a rapid resolution of the fever, and the diagnosis of scrub typhus was confirmed by a positive scrub IgM ELISA. HLH with a leukemoid reaction secondary to scrub typhus has not previously been reported in early infancy.
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PMID:Hemophagocytic lymphohistiocytosis with a leukemoid reaction in an infant with scrub typhus. 2612 73

Disseminated tuberculosis is an important differential diagnosis for fever of unknown origin (FUO) and it can present with hepatosplenomegaly and lymphadenopathy and may have meningitis and with hematological abnormalities including pancytopenia or a leukemoid reaction. We report the case of a 13-year old male who presented with fever, weight loss, pallor and massive splenomegaly with pancytopenia, in whom a bone marrow trephine biopsy showed caseating granulomata, who responded well to antituberculous treatment and has remained healthy on follow up after nine years.
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PMID:Extra pulmonary tuberculosis presenting as fever with massive splenomegaly and pancytopenia. 2705 79

Disseminated tuberculosis (DTB) often presents with protean clinical manifestations that often leads to potential diagnostic dilemmas. The nonspecific features may include pyrexia of unknown origin, hepatosplenomegaly, lymphadenopathy, meningitis, and a variety of hematological abnormalities, namely anemia, pancytopenia, and leukemoid reaction. Tuberculosis is one of the nonhematopoietic diseases that has been reported in conjunction with myelofibrosis. We, hereby, report a case of DTB with massive splenomegaly, severe pancytopenia, and marrow fibrosis.
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PMID:Myelofibrosis: An unusual presentation of disseminated tuberculosis. 3247 48


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