Rescue splenic artery embolization in an adult patient of sickle cell disease presented with acute splenic sequestration crisis.

BACKGROUND: Splenic sequestration crisis is a potentially fatal complication of sickle cell disease, mainly seen in young children. Only a few case series describe the acute splenic sequestration crisis in adults and its management, which primarily consists of supportive care and, in some cases, splenectomy. Splenic artery embolization has seldom been described in sickle cell disease. This is probably the first case in which an adult with sickle cell disease presented with an acute splenic sequestration crisis was managed successfully through splenic artery embolization. RESULTS: This 22-year-old female, a known case of sickle cell disease, presented with severe pain in the abdomen and low-grade intermittent fever for two days, secondary to an acute splenic sequestration crisis. The diagnosis of acute splenic sequestration was made based on clinical and blood parameters, ultrasonography, and computed tomography. Even with adequate supportive care and blood transfusions, the patient's condition worsened with a rapid fall in the hemoglobin and total platelet count. Considering splenectomy to be a high-risk procedure for this patient, a decision of rescue splenic artery embolization was taken, which was successful. CONCLUSION: Splenic artery embolization may be considered a lifesaving procedure in patients with acute splenic sequestration, where the risk of splenectomy can be high. Adequate post-procedure supportive care is vital for preventing complications.

Edwardsiella tarda Causing Fishbone Injury Cellulitis Leading to Sepsis in a Case of Hematological Malignancy-A Rare Report and Review of Literature.

Edwardsiella tarda ( E. tarda ), a gram-negative bacillus, a member of order Enterobacterales , is typically a fish pathogen frequently isolated from fresh and brackish water environments. It is very rarely implicated in human infections such as gastroenteritis (most common), cellulitis, gas gangrene, hepatobiliary infections, peritonitis, empyema, and meningitis. Bacteremia/sepsis caused by E. tarda can be fatal in humans, although very rare (<5%). To date, very few cases of E. tarda sepsis have been reported worldwide including India. We report a rare case of cellulitis caused by E. tarda following fishbone injury in a patient with underlying hematological malignancy leading to sepsis.

Core-binding factor abnormalities involving chromosome 16 in acute myeloid leukaemia: prognostic and therapeutic implications.

Core-binding factor (CBF) abnormality-associated myeloid neoplasms incorporate acute myeloid leukaemia (AML) (CBF-AML) with translocation t(8;21)(q22;q22.1) (AML1/ETO fusion) and inv(16)(p13.1q22) or translocation t(16;16)(p13.1;q22) (CBFB/MYH11 fusion) abnormalities which confer a favourable prognosis following cytarabine-based induction chemotherapy. Accumulating evidence from the molecular studies have stratified CBF-AML into favourable and unfavourable subgroups based on the associated cooperating mutations that impact the outcome. We describe a case of acute myelomonocytic leukaemia with abnormal eosinophils (M4Eo) in a woman in her 20s who was found to have CBFß/MYH11 fusion along with mutated c-KIT (exon 17) and KRAS (exon 2) genes by next-generation sequencing. She had an aggressive clinical course following initiation of cytarabine-based induction chemotherapy. The underlying mutational landscape may significantly influence the biological behaviour of otherwise favourable risk of CBF-AML cases.

Usefulness of Leucocyte Cell Population Data by Sysmex XN1000 Hematology Analyzer in Rapid Identification of Acute Leukemia.

Leukocyte cell population data (CPD) generated by hematology auto analyzers are reported to be useful in screening of sepsis patients. However, there is a paucity of literature highlighting the utility of CPD in screening of acute leukemias (AL). Leucocyte CPD obtained by Sysmex XN1000 hematology analyzer from 210 cases of ALs [22 acute promyelocytic leukemia (APL), 79 non-APL acute myeloid leukemia (non-APL-AML) and 109 acute lymphoblastic leukemia (ALL)] were compared with 100 healthy and 52 reactive controls. Receiver operator curves were drawn to determine the cut-off values of individual parameters. The regression equations combining the best parameters were then formulated to calculate a cut-off value for discrimination among AL subgroups and controls. Acute leukemias showed significant differences (p < 0.05) in various CPD parameters compared to control subjects. A combination of best CPD parameters discriminated ALs from healthy controls (cut off; 0.443, sensitivity of 94% and specificity of 91%), ALs from reactive controls (cut off; 0.576, sensitivity; 97%, specificity; 92%), APL from non-APL-AML (cut off; 0.174, sensitivity of 91% and specificity of 67%), and AML from ALL (cut off; 1.338, sensitivity; 86.1%, specificity; 75%). The CPD from Sysmex XN 1000 analyzer could be a useful tool in screening and lineage characterization of acute leukemias; particularly at centers where high-end technical expertise is still not available. Supplementary Information: The online version contains supplementary material available at 10.1007/s12288-021-01488-9.

A rare maxillofacial manifestation of acute lymphoblastic leukemia in a 9-year-old child.

Acute lymphoblastic leukemia (ALL) is considered as the most common malignant neoplasm of childhood and the frequent cause of death from cancer before 20-years of age. The facial swelling mimicking a maxillofacial tumor is rarely associated with ALL. Clinicians should be aware of such rare manifestation of ALL. We present a case with an atypical mass in the facial region secondary to ALL, which resulted in diagnostic dilemma. Reports of such atypical swelling in patients with ALL are occasional. The swelling was aggressive and the disease had a fulminant course.

Bone marrow trephine immunohistochemistry is useful in characterizing malignancy-associated myelonecrosis: A retrospective observational study.

OBJECTIVE: We aim to describe the utility of immunohistochemistry (IHC) in characterizing malignancy-associated myelonecrosis (MN) on bone marrow trephine biopsies (BMBx) as a part of initial workup. MATERIALS AND METHODS: Patten and intensity of antigenic immunoexpression in necrotic tumor cells on BMBx were evaluated in a series of cases using standardized avidin-biotin-complex immunoperoxidase technique after heat-induced epitope retrieval and compared the same with viable tumor cells wherever available. RESULTS: Fifteen out of 2494 (0.6%) cases (median age: 28 years; range: 4 to 66 years) had evidence of MN (extensive in eight, moderate in five, and focal in two) secondary to hematological (N = 9) and solid (N = 6) malignancies. Five (33.3%) had pancytopenia, and eight (53.3%) had difficult and/or hemodiluted aspirate. Antigenic expression for CD10, CD79a, CD3, CD7, and CD20 was retained by necrotic leukemic blasts or lymphoma cells; CD34, TdT, and PAX5 showed heterogeneous expression; and a weak Golgi zone (dot like) CD30 positivity was noted in Reed-Sternberg (RS) or RS-like giant cells. Necrotic epithelial metastases retained pancytokeratin in all and showed variable positivity for prostate-specific antigen, carcinoembryonic antigen, CK20, ER, PR, and GATA3. Necrotic neuroblastomas (N = 2) retained positivity for synaptophysin and chromogranin, whereas retained nuclear positivity for NKX2.2 in necrotic Ewing family of tumor (N = 1) aided in early diagnosis. CONCLUSION: Myelonecrosis may retain tumor antigenicity, and immunohistochemistry using selected panel of antibodies should be tried in such challenging cases for an early presumptive diagnosis and further decision making.