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Saika Somjee

Publications and source records attributed to Saika Somjee.

6 recordsLinked to original sources
PubMed2004

Diagnosis and characterization of Hb C/Hb Iowa: a rare but easily misidentified compound heterozygous condition.

Hb Iowa is a rare hemoglobin (Hb) variant with a Gly --> Ala substitution at position 119 of beta-globin. It was previously reported only in an African American infant who was also heterozygous for Hb S [beta6(A3)Glu --> Val] and her mother (Hb A/Iowa). Here we describe the second report of Hb Iowa, the first in conjunction with Hb C [beta6(A3)Glu --> Lys]. The patient was an African American girl, originally diagnosed as homozygous Hb C during neonatal screening. When seen in our clinic, hematological data for both the child and her mother (Hb C trait) indicated mild anemia with slightly low mean corpuscular volume (MCV) but normal red blood cell (RBC) count. Analysis of blood from the child by capillary isoelectric focusing (cIEF) identified Hb C and an unknown Hb variant with an isoelectric point (pI) intermediate to that of Hbs F and A. The unknown variant was identified as Hb Iowa by DNA sequence analysis of the beta-globin gene (GGC --> GCC). Both reported cases of Hb Iowa indicated that there are no abnormal hematological manifestations associated with this rare Hb variant. In both cases, however, Hb Iowa was mistaken for Hb F during routine neonatal screening by high performance liquid chromatography (HPLC) and/or gel IEF. Neonatal misidentification of Hb Iowa led to misdiagnosis of sickle cell disease and Hb C disease, respectively. In our patient, Hb Iowa was also misidentified as Hb A at 2 years of age by a commercial reference laboratory using cellulose acetate and citrate agar gel electrophoresis. This led to an incorrect diagnosis of Hb C trait. These results show that commonly used analytical methods can easily misidentify Hb Iowa as Hbs F or A in neonates, or older individuals, resulting in incorrect identification of the Hb phenotype. We conclude that the presence of Hb Iowa, or other variants with similar pIs, should be considered in cases where the results of follow-up testing conflict with neonatal diagnosis of sickle cell or Hb C disease, or where clinical presentation does not agree with diagnosis of either homozygous or heterozygous Hb S or Hb C.

Alleles↗
PubMed2003

Benign multilocular cystic nephroma.

This article reports a case of cystic nephroma to bring awareness about the benign nature of this condition. The patient presented with a painless abdominal mass. Computed tomography showed a homogeneous, multicystic tumor of the superolateral portion of the left kidney with thin septa without solid parts. Histology confirmed the diagnosis of cystic nephroma.

Female↗
PubMed2002

Leukemia in infants.

OBJECTIVE: Acute Leukemia is rare in infants. It is characterized by non-specific symptomatology requiring a high index of suspicion on the part of a pediatrician for referral and diagnosis. It has peculiar biological features, unresponsiveness to treatment and poor prognosis. METHODS: Eighteen infants with acute leukemia were seen during 1994 to 2001 and were analyzed on the basis of clinical and laboratory data. There were 13 cases of Acute Lymphoblastic Leukemia (ALL), 4 cases of Acute Myeloid Leukemia (AML) and one case remained unclassifiable, as the surface markers could not be done. Morphologically 9/13 cases of ALL were of FAB L1 type and remaining of L2 subtype, and 2/4 cases of AML were of FAB M1 type and remaining of M2 subtype. RESULT: Clinical data was available completely only in 11 cases. Hyperleucocytosis was present in 4 cases, organomegaly in 8 cases and lympadenopathy in 5 cases. One patient presented with a chloroma in the retrorbital region although there was no parenchymal involvement of the brain. Immunophenotyping could be done in 13 cases, where 7 cases were diagnosed as CALLA positive-ALL (HLADR+, CD19+, CD10+), 2 cases as Early Pre-B ALL (HLADR+, CD19+, CD10 negative), one as T ALL (cCD3+, CD2+, CD7+) and 3 cases as AML (CD13+, CD33+, HLADR+). None of our patients received treatment.

Female↗
PubMed2002

Passive active prophylaxis against Hepatitis B in children with acute lymphoblastic leukemia.

The aim of this study was to assess the antibody response to combined passive active immunisation versus active immunisation along with interferon against Hepatitis B in 60 patients with acute lymphoblastic leukemia (ALL) between 1 and 21 years of age with negative Hepatitis B virus (HBV) serology at presentation. Thirty-one patients received combined passive active immunisation with human specific Hepatitis B immunoglobulin (HEPABIG-VHB Pharmaceuticals) and Hepatitis B vaccine (arm I) and 29 patients received active immunisation along with interferon (arm II). Protective antibody levels were detected in 89.6 and 21% patients, respectively, at the 6-month evaluation. Infection with HBV occurred in 17 and 59% patients, respectively, at the 6-month evaluation. Interferon, thus, failed to serve the role as a vaccine adjuvant. At the 9-month evaluation of patients who received immunoglobulin, protective antibody titers were lost in 8 out of 19 evaluable patients (42%) and of these, 3 patients became HBsAg reactive at this point of time. This study indicated that 47.3% patients undergoing aggressive chemotherapy responded to combined passive active prophylaxis with protective titers of antiHBs at the 9-month evaluation. However, the rate of HBV infection was greatly reduced to 27%. We suggest that usage of passive immunisation in the aggressive phase, followed by active immunisation after cessation of intense chemotherapy would be a better option to increase the rates of protective antibody levels in these immunocompromised patients with leukemia.

Acute Disease↗