Patience Pays Off: A Case of Self Exfoliation of Large Parotid Sialolith

Sialolithiasis is one of the common diseases affecting the salivary glands. The submandibular gland is the most commonly affected gland among the three major salivary glands. The incidence of parotid sialolith is rare and when present, often demands surgical removal. Here, we report a rare case of a female patient presented with large sialolith indicated for surgical removal. However, surprisingly, it resulted in self-exfoliation eliminating the need for surgery as the patient had opted to wait.

Chronic myeloid leukemia in case of Klinefelter syndrome.

Klinefelter syndrome (KS) is a sex chromosome disorder and has been reported to be associated with increased risk for malignancies. We report a 22‑year‑old male patient who was diagnosed to have chronic myeloid leukemia in chronic phase. Bone marrow cytogenetic examination revealed karyotype 47, XXY, t (9; 22)(q34, q11) suggestive of KS with presence of Philadelphia chromosome. The patient was treated with oral imatinib mesylate (400 mg/day). Complete hematological response was achieved after 2 months of therapy. The bcr‑abl/abl transcript percentage measured from peripheral blood at baseline, 1 and 2 years after imatinib were 97%, 1.99%, 0.007%, respectively. He remains in complete hematological and major molecular remission after 2 years of continued imatinib therapy.

Splenic lymphoma with villous lymphocytes.

Splenic lymphoma with villous lymphocytes (SLVL) is a rare disorder that comprises less than 1% of lymphoid neoplasms. It is the leukemic counterpart of splenic marginal zone lymphoma (SMZL) and is characterized by splenomegaly, often with no lymphadenopathy, moderate lymphocytosis and villous lymphocytes on peripheral blood smear. Here, we report a case of SLVL in a 56-year-old male with very high leukocyte counts, massive splenomegaly and relatively few leukemic cells with subtle villous projections on the surface. This disorder is often confused with other chronic lymphoproliferative disorders, especially chronic lymphocytic leukemia (CLL) and hairy cell leukemia and should be differentiated from them. We are reporting this case to highlight the diagnostic pitfalls associated with this disorder.

Immunophenotypic patterns in precursor T-cell neoplasm.

T-cell lymphoproliferative disorders are a heterogeneous group of lymphoid neoplasm that can mimic both benign conditions and non-hematopoietic tumors. In routine clinical practice, morphology and immunophenotyping forms the basis of their diagnosis. In this retrospective analysis, we evaluate the utility of flow cytometric immunophenotyping patterns in diagnosis of precursor T-cell neoplasm. Aberrant expression of T-cell antigens was found in all the cases of precursor T-cell neoplasm. The residual normal T-lymphocytes, identifiable in majority of cases, were found to be useful in evaluation of quantitative differences in antigen expression by leukemic cells. A careful analysis of flow cytometric immunophenotyping data can provide additional information which is useful for diagnosis of precursor T-cell neoplasm. This information can be further utilized for analysis of minimal residual disease in these tumors.

Profile of liver involvement in dengue virus infection.

BACKGROUND: Data are scarce on liver involvement in adult patients with dengue virus infection. METHODS: During a recent outbreak of dengue fever in Uttar Pradesh, India, we looked for evidence of liver dysfunction among patients with dengue fever. RESULTS: A total of 45 patients with dengue fever (age 7-65 [median 33] years; 29 men; 39 adults) were studied, including 23 with uncomplicated dengue fever, 15 with dengue haemorrhagic fever and 7 with dengue shock syndrome. The median platelet count was 34 x 10(9)/L (9-99 x 10(9)). Seven patients (15%) had jaundice, 11 (24%) hepatomegaly and 9 clinically detectable ascites; none had splenomegaly. Twelve patients (30%) had hyperbilirubinaemia. Serum alanine and aspartate aminotransferase activities were elevated in 43 patients (96%) each; 5-fold elevated levels were more frequent in severe disease. Hypoalbuminaemia was found in 31/41 patients (76%). Seven patients died, including 2 with acute liver failure. CONCLUSION: Our data show that liver injury is common in adult patients with dengue infection. Further studies are needed to determine the mechanism of liver injury in this disease.

Combined occurrence of von Willebrand's disease and factor XIII deficiency: a case report.

We report the case of a three year old female child with combined occurrence of von Willebrand's disease and factor XIII deficiency, an extremely rare combination. The patient presented with prolonged bleeding following cuts and wounds. Clot solubility test using 5M urea was positive. Platelet aggregation using ristocetin was reduced, which corrected on adding normal plasma. Aggregation with other agonists was normal. We discuss the clinico- hematological profile of the case. Only one such case has been reported in literature in the past to the best of our knowledge.