MyMedR

Fulltext Dengue death with evidence of hemophagocytic syndrome and dengue virus infection in the bone marrow

Ab-Rahman HA, Wong PF, Rahim H, Abd-Jamil J, Tan KK, Sulaiman S, et al.

Springerplus, 2015;4:665.

PMID: 26558168 DOI: 10.1186/s40064-015-1463-z

INTRODUCTION: HPS is a potentially life-threatening histiocytic disorder that has been described in various viral infections including dengue. Its involvement in severe and fatal dengue is probably more common but is presently under recognized.
CASE DESCRIPTION: A 38-year-old female was admitted after 5 days of fever. She was deeply jaundiced, leukopenic and thrombocytopenic. Marked elevation of transaminases, hyperbilirubinemia and hypoalbuminemia were observed. She had deranged INR values and prolonged aPTT accompanied with hypofibrinogenemia. She also had splenomegaly. She was positive for dengue IgM. Five days later she became polyuric and CT brain image showed gross generalized cerebral edema. Her conditions deteriorated by day 9, became confused with GCS of 9/15. Her BMAT showed minimal histiocytes. Her serum ferritin level peaked at 13,670.00 µg/mL and her sCD163 and sCD25 values were markedly elevated at 4750.00 ng/mL and 4191.00 pg/mL, respectively. She succumbed to the disease on day 10 and examination of her tissues showed the presence of dengue virus genome in the bone marrow.
DISCUSSION AND EVALUATION: It is described here, a case of fatal dengue with clinical features of HPS. Though BMAT results did not show the presence of macrophage hemophagocytosis, other laboratory features were consistent with HPS especially marked elevation of ferritin, sCD163 and sCD25. Detection of dengue virus in the patient's bone marrow, fifteen days after the onset of fever was also consistent with the suggestion that the HPS is associated with dengue virus infection.
CONCLUSIONS: The findings highlight HPS as a possible complication leading to severe dengue and revealed persistent dengue virus infection of the bone marrow. Detection of HPS markers; ferritin, sCD163 and sCD25, therefore, should be considered for early recognition of HPS-associated dengue.
KEYWORDS: Bone marrow; Dengue; Ferritin; Hemophagocytic syndrome; MAS; Macrophage
Study site: University Malaya Medical Centre (UMMC), Kuala Lumpur, Malaysia

Matched MeSH terms: Fatal Outcome

Fatal septicemic shock associated with Strongyloides stercoralis infection in a patient with angioimmunoblastic T-cell lymphoma: a case report and literature review

Abdelrahman MZ, Zeehaida M, Rahmah N, Norsyahida A, Madihah B, Azlan H, et al.

Parasitol Int, 2012 Sep;61(3):508-11.

PMID: 22575692 DOI: 10.1016/j.parint.2012.04.005

Strongyloides stercoralis infection can persist in the host for several decades, and patients with cancer and other clinical conditions who are exposed to immunosuppressive therapy are at risk of developing hyperinfection.

Matched MeSH terms: Fatal Outcome

Fulltext Metastatic melanoma: a rare cause of central airway obstruction

Abdul Hamid MF, Ban Yu-Lin A, Hassan TM, Mohammad N

BMJ Case Rep, 2017 Nov 04;2017.

PMID: 29103009 DOI: 10.1136/bcr-2017-221545

A middle-aged woman with recurrent malignant melanoma presented initially with massive left pleural effusion. There was a complete obliteration of the left main bronchus on flexible bronchoscopy caused by a mass. Serial cryo-debulking of the tumour was done under rigid bronchoscopy; however, the outcome was not favourable due to the aggressive tumour growth. Vemurafenib was planned after thoracic radiation. She was not keen for the biologics treatment due to financial constraints. We report a case of central airway obstruction due to recurrent aggressive melanoma. More evaluations are needed on the role of interventional pulmonologist for bronchoscopic debulking of this rapidly growing tumour as well as the role of biological agents in treating such cases.

Matched MeSH terms: Fatal Outcome

Fulltext Malignant rhabdoid tumour of the brain

Abdullah JM, Rahman ZA, Ariff AR, Jaafar H, Phang KS

Singapore Med J, 2004 Jun;45(6):286-8.

PMID: 15181525

Rhabdoid tumour is a rare childhood tumour with poor prognosis. We report a 13-month-old Malay girl suffering from this tumour that was located at the left fronto-temporo-parietal region of the brain. Computed tomography showed a large irregular enhancing mass that caused obstructive hydrocephalus. The tumour did not reduce in size after three operations and finally the patient succumbed to the disease four months after diagnosis.

Matched MeSH terms: Fatal Outcome

Loss of weight in a female heavy smoker with diffuse interstitial pulmonary fibrosis

Al-Adsani A, Dahniya MH, Al-Adsani N

Postgrad Med J, 2001 Feb;77(904):127, 137-8.

PMID: 11161092

Matched MeSH terms: Fatal Outcome

Fatal necrotizing pneumonia caused by Panton-Valentine leukocidin-producing hospital-acquired Staphylococcus aureus: a case report

Al-Talib H, Hasan H, Yean CY, Al-Ashwal SM, Ravichandran M

Jpn J Infect Dis, 2011;64(1):58-60.

PMID: 21266757

Panton-Valentine leukocidin (PVL) is a cytotoxin which causes leukocyte destruction and tissue necrosis. Although it is produced by fewer than 5% of Staphylococcus aureus strains, PVL-producing S. aureus is emerging as a serious problem worldwide. There has been a marked increase in the incidence of necrotizing lung infections with a very high mortality associated with these strains. This report describes a fatal case of hospital-acquired necrotizing pneumonia caused by PVL-positive methicillin-susceptible S. aureus in a patient with a brain tumor.

Matched MeSH terms: Fatal Outcome

Fulltext Systematic Epstein-Barr virus-positive T-cell lymphoproliferative disease presenting as a persistent fever and cough: a case report

Ameli F, Ghafourian F, Masir N

J Med Case Rep, 2014;8:288.

PMID: 25163591 DOI: 10.1186/1752-1947-8-288

Systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease is an extremely rare disorder and classically arises following primary acute or chronic active Epstein-Barr virus infection. It is characterized by clonal proliferation of Epstein-Barr virus-infected T-cells with an activated cytotoxic phenotype. This disease has a rapid clinical course and is more frequent in Asia and South America, with relatively few cases being reported in Western countries. The clinical and pathological features of the disease overlap with other conditions including infectious mononucleosis, chronic active Epstein-Barr virus infection, hemophagocytic lymphohistiocytosis and natural killer cell malignancies. We describe the rare case of systemic Epstein-Barr virus-positive T-cell lymphoproliferative childhood disease in a 16-year-old Malay boy.

Matched MeSH terms: Fatal Outcome

Fulltext Clear cell adenocarcinoma of the cervix in a ten-year-old girl without prenatal diethylstilbestrol exposure

Andi Asri AA, Lim BK, Lim YK, A Latiff L

Singapore Med J, 2016 Aug;57(8):470.

PMID: 27549741 DOI: 10.11622/smedj.2016138

Matched MeSH terms: Fatal Outcome

Fulltext A very rare and rapidly fatal case of Chromobacterium violaceum septicemia

Ang YM

Med J Malaysia, 2004 Oct;59(4):535-7.

PMID: 15779589

Chromobacterium violaceum infection is rare but causes a high mortality rate particularly in immunosuppressed persons. Since its clinical presentation is non-specific and the diagnosis basically relies upon blood culture and sensitivity, this infection should be considered among the organisms targeted empirically for antibiotic therapy when a cellulitis or rapidly progressive illness follows exposure to water or soil. This is a case of fulminant septicemia caused by this rarely encountered organism.

Matched MeSH terms: Fatal Outcome

Li-Fraumeni syndrome in a Malaysian kindred

Ariffin H, Martel-Planche G, Daud SS, Ibrahim K, Hainaut P

Cancer Genet. Cytogenet., 2008 Oct;186(1):49-53.

PMID: 18786442 DOI: 10.1016/j.cancergencyto.2008.06.004

We report on a Malaysian kindred with Li-Fraumeni syndrome. The proband was an 8-year-old girl who presented with embryonal rhabdomyosarcoma of the trunk at the age of 8 months and developed a brain recurrence at the age of 7 years, which was 5 years after remission. A younger sister later developed adrenocortical carcinoma at the age of 6 months. Their mother and maternal grandmother were diagnosed with breast cancer at the ages of 26 and 38 years, respectively. TP53 mutation detection in this family revealed a duplication of a GGCGTG motif starting at nucleotide 17579 in exon 10, resulting in an in-frame insertion of two amino acids between residues 334 and 336 in the tetramerization domain of the p53 protein. This mutation was found in the proband and her affected sister as well as her mother. In addition, the mutation was detected in two other siblings (a brother aged 3 years and a sister aged 18 months) who have not yet developed any malignancy. Sequencing of TP53 in the father and two other asymptomatic siblings revealed wild-type TP53. To our knowledge, this is a first report of a Li-Fraumeni syndrome family in Southeast Asia.

Matched MeSH terms: Fatal Outcome

Secondary B-cell acute lymphoblastic leukemia following Wilms' tumor: clinical and in vitro chemosensitivity studies

Ariffin H, Muthukkumaran T, Stanslas J, Sabariah AR, Veerasekaran N, Lin HP

Leuk Lymphoma, 2005 Aug;46(8):1233-7.

PMID: 16085568

We report the clinical features and in vitro chemosensitivity assay findings of a 13-year-old girl who developed secondary B-cell acute lymphoblastic leukemia (ALL) 7 years after a diagnosis of Wilms' tumor. The patient was treated using the Berlin - Frankfurt - Muenster (BFM) ALL chemotherapy protocol with poor response to initial therapy before succumbing to sepsis. An in vitro chemosensitivity assay on her peripheral blood lymphoblasts was performed while she was undergoing induction therapy and showed a high level of resistance to drugs commonly used for ALL therapy, e.g. steroids, anthracyclines, vincristine and L-asparaginase. The mechanism of chemoresistance was not elicited, but was probably not related to P-glycoprotein (P-gp) over-expression. We believe that the in vitro chemosensitivity assay is a good indicator of cellular response to chemotherapy and may provide reliable information for the basis of the selection of drugs to be used for the treatment of similarly rare patients rather than relying on "standard" protocols.

Matched MeSH terms: Fatal Outcome

Fulltext Intraabdominal desmoplastic small round cell tumour in an 11-year-old boy

Ariffin H, Ariffin WA, Wong KT, Ramanujam TM, Lin HP

Singapore Med J, 1997 Apr;38(4):169-71.

PMID: 9269398

A case of desmoplastic small round cell tumour (DSRCT) is presented. This aggressive and rare neoplasm predominantly affects males and is almost exclusively intraabdominal in location. It is unique in that neural, mesenchymal and epithelial markers are co-expressed. Despite multi-modal therapy, the prognosis is extremely poor. The present report details the clinical features and typical pathological findings of DSRCT in an 11-year-old boy, who succumbed to the disease 16 months after diagnosis despite multiple chemotherapeutic regimes.

Matched MeSH terms: Fatal Outcome

Infantile malignant hemangiopericytoma of the orbit

Arshad AR, Normala B

Ophthalmic Plast Reconstr Surg, 2008 3 22;24(2):147-8.

PMID: 18356724 DOI: 10.1097/IOP.0b013e31816746b4

A 5-month-old girl presented with a soft-tissue mass over the lateral aspect of her right eyebrow. Surgical resection revealed a diagnosis of infantile malignant hemangiopericytoma. The mass recurred after excision and reconstruction. The patient died 4 months later with brain and lung metastasis.

Matched MeSH terms: Fatal Outcome

Solitary sebaceous nevus of Jadassohn complicated by squamous cell carcinoma and basal cell carcinoma

Arshad AR, Azman WS, Kreetharan A

Head Neck, 2008 Apr;30(4):544-8.

PMID: 17972311 DOI: 10.1002/hed.20708

BACKGROUND: Sebaceous nevus is a benign congenital epidermal nevus. Its association with basal cell carcinoma is well known.
METHOD: This is a case report of sebaceous carcinoma complicated by both basal cell carcinoma and squamous cell carcinoma.
RESULTS: The behavior of this tumor is very aggressive, resulting in poor prognosis.
CONCLUSIONS: All sebaceous nevi should be excised early.

Matched MeSH terms: Fatal Outcome

Post partum splenic artery aneurysm rupture

Asokan S, Chew EK, Ng KY, Thanaletchimy N, Asmiati A, Kong NM

J Obstet Gynaecol Res, 2000 Jun;26(3):199-201.

PMID: 10932982

Ruptured splenic artery aneurysm during pregnancy is a rare event with high maternal and fetal mortality rate. A case of ruptured splenic artery aneurysm in the post partum period is presented. The literature is reviewed on pathophysiology, clinical presentation and management of this rare and potentially fatal entity.

Matched MeSH terms: Fatal Outcome

Fulltext Molar pregnancy with normal viable fetus presenting with severe pre-eclampsia: a case report

Atuk FA, Basuni JBM

J Med Case Rep, 2018 May 21;12(1):140.

PMID: 29779493 DOI: 10.1186/s13256-018-1689-9

BACKGROUND: While gestational trophoblastic disease is not rare, hydatidiform mole with a coexistent live fetus is a very rare condition occurring in 0.005 to 0.01% of all pregnancies. As a result of the rarity of this condition, diagnosis, management, and monitoring will remain challenging especially in places with limited resources and expertise. The case we report is an interesting rare case which presented with well-described complications; only a few similar cases have been described to date.

CASE PRESENTATION: We report a case of a 21-year-old local Sarawakian woman with partial molar pregnancy who presented with severe pre-eclampsia in which the baby was morphologically normal, delivered prematurely, and there was a single large placenta showing molar changes.

CONCLUSION: Even though the incidence of this condition is very rare, recognizing and diagnosing it is very important for patient care and it should be considered and looked for in patients presenting with pre-eclampsia.

Matched MeSH terms: Fatal Outcome

Fulltext Juvenile myelomonocytic leukaemia: a case series

Azma RZ, Zarina AL, Hamidah A, Jamal R, Sharifah NA, Ainoon O, et al.

Malays J Pathol, 2009 Dec;31(2):121-8.

PMID: 20514855 MyJurnal

Juvenile myelomonocytic leukaemia (JMML), previously known as juvenile chronic myeloid leukaemia (JCML) is a rare, myelodysplastic - myeloproliferative disease typically presenting in early childhood. This disorder is difficult to distinguish from other myeloproliferative syndrome such as chronic myeloid leukaemia (CML) because of the similarities in their clinical and bone marrow findings. However, because of its unique biological characteristics such as absolute monocytosis with dysplasia, absence of Philadelphia chromosome or BCR-ABL fusion protein, hypergammaglobulinaemia and raised fetal haemoglobin level, this disorder does not satisfy the criteria for inclusion in the CML or chronic myelomonocytic leukaemia (CMML) group, as seen in adult patients. We describe a series of three patients with JMML, who had almost similar clinical and laboratory findings, and discuss the difficulty in the classification and treatment of the disease.

Matched MeSH terms: Fatal Outcome

Mutations in mitochondrial complex I assembly factor NDUFAF3 cause Leigh syndrome

Baertling F, Sánchez-Caballero L, Timal S, van den Brand MA, Ngu LH, Distelmaier F, et al.

Mol Genet Metab, 2017 03;120(3):243-246.

PMID: 27986404 DOI: 10.1016/j.ymgme.2016.12.005

NDUFAF3 is an assembly factor of mitochondrial respiratory chain complex I. Variants in NDUFAF3 have been identified as a cause of severe multisystem mitochondrial disease. In a patient presenting with Leigh syndrome, which has hitherto not been described as a clinical feature of NDUFAF3 deficiency, we identified a novel homozygous variant and confirmed its pathogenicity in patient fibroblasts studies. Furthermore, we present an analysis of complex I assembly routes representative of each functional module and, thereby, link NDUFAF3 to a specific step in complex I assembly. Therefore, our report expands the phenotype of NDUFAF3 deficiency and further characterizes the role of NDUFAF3 in complex I biogenesis.

Matched MeSH terms: Fatal Outcome

A case of T-cell acute lymphoblastic leukemia after treatment of acute promyelocytic leukemia

Bee PC, Gan GG, Sangkar JV, Teh A, Goh KY

Int J Hematol, 2004 May;79(4):358-60.

PMID: 15218965

We diagnosed T-cell acute lymphoblastic leukemia (T-ALL) with multiple cytogenetic abnormalities in a 17-year-old girl a year after she had received a diagnosis of acute promyelocytic leukemia (APML). After the diagnosis of APML in June 2001, the patient was treated with idarubicin and all-trans-retinoic acid. In September 1999, her younger sister also received a diagnosis of APML and to date has remained well. T-ALL after remission of APML is very rare, and only 1 such case has been reported. Possible causes include therapy-related reasons, genetic susceptibility to leukemia, and environmental exposure.

Matched MeSH terms: Fatal Outcome

Unexpected infant death secondary to a pulmonary infiltration due to acute myelocytic leukaemia

Ben Khelil M, Chkirbene Y, Mlika M, Haouet S, Hamdoun M

Malays J Pathol, 2017 Aug;39(2):193-196.

PMID: 28866704

Acute myeloid leukaemia (AML) often presents with non-specific symptoms such as fatigue, anaemia or infection. Pulmonary involvement is uncommon in AML during the course of the disease and is usually caused by infection, haemorrhage, leukaemic pulmonary infiltrates and leukostasis. Lung localization of AML is very uncommon and potentially life threatening if not diagnosed and treated rapidly. The authors describe the sudden death of an asymptomatic five-month-infant because of a misdiagnosed lung localization of AML. Autopsy examination followed by histopathological studies showed an extensive leukostasis and extramedullary leukaemic infiltrating the lungs. Special stains and immunohistochemical studies revealed findings consistent with acute myelogenous leukaemia. This case suggests that underlying acute leukaemia should be considered as a cause of flu-like symptoms in infants. Medical personnel are urged to be alert to fever, sore throat, weakness and dyspnea that may be characteristic of serious systemic diseases.

Matched MeSH terms: Fatal Outcome

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