Diagnostic Evaluation of Polycythemia Vera at High Altitude.

BACKGROUND: Polycythemia is a common medical problem, frequently acquired and reactive to secondary conditions. High-altitude-associated hypoxia contributes to the greater prevalence of polycythemia at altitude. Primary clonal polycythemia vera (PV), even though it is rare, requires a different therapeutic approach. Suspicion of PV usually drives the diagnostic workup of polycythemia. METHODS: In this retrospective lab record study, we collected all JAK2 tests requested over a three-year period. We analyzed requests that were made for the evaluation of polycythemia. Complete blood count (CBC) and imaging of the abdomen were collected. RESULTS: Out of 208 total requests, 136 were for the purpose of polycythemia evaluation. JAK2 mutation was positive (confirming the presence of PV) in 22 (16.7%) cases. PV patients have the usual demographics reported elsewhere. Additionally, PV patients exhibit distinct hemogram results featuring leukocytosis, thrombocytosis, and hypochromic microcytic red blood cells (RBCs) related to the associated iron deficiency. CONCLUSIONS: Many patients with polycythemia at altitude might be unnecessarily considered for an evaluation of PV, if hemoglobin/hematocrit is the sole deciding criterion. PV patients have a distinct CBC pattern that can be exploited to better select patients with polycythemia for further evaluation and thus reduce unnecessary workups.

Clinical Impact of Percentage of Natural Killer Cells and Natural Killer-Like T Cell Population in Acute Myeloid Leukemia.

BACKGROUND: Natural killer (NK) function defects have been seen in many hematological malignancies, including acute myeloid leukemia (AML). AML is associated with deficient human leukocyte antigen (HLA) expression on leukemia blasts which become targets for killing by NK and natural killer-like T (NKT) cells. However, NK and NKT cells are not effective in killing autologous leukemia blasts, maybe due to number or functional abnormalities. The aim of the work was to detect the number and percentage of NK and NKT cells in patients with AML and the impact of their percentage on the prognosis, response to treatment and survival. METHODS: Bone marrow and peripheral blood samples were collected from 50 adult patients diagnosed as de novo AML who presented to the Hematology Unit in the Oncology Center Mansoura University (OCMU) at time of diagnosis. NK and NKT cells were detected by using immunophenotyping by expression of cell surface and cytoplasmic markers (anti-CD3 fluorescein isothiocyanate (FITC), anti-CD16/56 phycoerythrin (PE)). RESULTS: We observed significant reduction in the median values of NK and NKT cells in AML patients in comparison to normal values. There was an insignificant correlation to response to induction treatment. While a significant correlation to overall survival (OS) (P = 0.03) was observed. The correlation to risk stratification was significant with NK cells (P < 0.001), but not with NKT cells (P = 0.23). CONCLUSION: We concluded that the number and percentage of NK and NKT cells decreased significantly in AML patients and the frequency of NK and NKT cells is inversely proportionate with prognosis and OS in studied AML patients. We recommend correlating both number and function of NK and NKT cells in future studies to help provide a wide field of interest for possibility of demonstrating novel therapies using NK cells for curing AML.

Multidetector computed tomography evaluation of synchronous lymphoma and other solid malignancies.

OBJECTIVE: The objective of this study is to review the multidetector computed tomography (MDCT) findings of synchronous lymphoma and other solid malignancies. PATIENTS AND METHODS: This retrospective study included 18 patients confirmed with diagnosis of lymphoma and other solid malignancies. They were 8 women and 10 men (mean age, 62.5 year; range, 44-73 years). CT scanning was performed on one of the two systems: 64 MDCT in 11 patients and 6 MDCT in 7 patients. All 36 malignancies were underwent pathological evaluation. RESULTS: All cases were confirmed pathologically. Lymphomas were Hodgkin disease ( n = 5 patients) and non-Hodgkin lymphoma ( n = 13 patients). Hepatocellular carcinoma was detected in five patients. Bronchogenic carcinoma was detected in two patients. Renal cell carcinoma was detected in two patients. Breast carcinoma was detected in two patients. Prostatic carcinoma was detected in two patients. Gastric carcinoma was detected in two patients. Endometrial carcinoma was detected in one patient. Colonic carcinoma was detected in one patient. Thyroid carcinoma was detected in one patient. CONCLUSIONS: MDCT scanning is accurately imaging modality for the evaluation of synchronous lymphoma and other solid malignancies. More reports and accumulation of such cases should help to clarify the mechanisms, contribute to a further understanding of this phenomenon, and may lead to a new treatment strategy for synchronous lymphoma and other solid malignancies.

MICROtransplant to refractory acute myeloid leukemia in Egyptian population.

PURPOSE OF REVIEW: The purpose of this review is to outline Egyptian experience of nonengraftment haploidentical cellular therapy [microtransplantation (MST)] for patients with refractory acute myeloid leukemia. RECENT FINDINGS: The use of granulocyte colony-stimulating factor primed halo-identical MST appears to be a biologically active therapy in patients with refractory acute myeloid leukemia (AML), especially in patients received less than four previous chemotherapy lines, fludarabine-free previous chemotherapy, response naïve and young age patients. SUMMARY: Refractory AML is still challenging. MST is promising, however the optimum conditioning, stem-cell dose, matching degree are factors should be optimized.

Unusual behavior of diffuse large B-cell lymphoma as cardiac invasion with rapid spontaneous regression after fine needle aspiration cytology.

OBJECTIVE: Spontaneous regression (SR) of lymphoma is a rare phenomenon. While the precise mechanism of SR remains unknown, apoptosis may be associated with its process. Here, we present a case of a 52-year-old woman was admitted to our hospital with cough and orthopnea for 2 weeks. Multi-detector computed tomography of whole body showed anterior and middle mediastinal soft tissue mass with multiple adjacent malignant lymphadenopathy. The mediastinal mass invaded right atrium and pericardium. Another left subphrenic retro-pancreatic mass was detected. This mass surrounded upper pole of left kidney. Fine needle aspiration cytology was done and revealed lymphocytic smear with advised tru-cut biopsy. CT guided tru-cut was taken after 8 days. During CT guided technique; marked regression of left subphrenic mass was detected. Post-contrast MDCT scan was done and revealed partial response of the masses after 8 days. The partial regressive course of this disease suggests the effectiveness of fine needle aspiration cytology in initiating spontaneous regression. Tru-cut biopsy revealed non-Hodgkin lymphoma (diffuse large B-cell type). We report a case of NHL with abnormal location and spontaneous regression.

A Metastatic Signet Ring Cell Carcinoma Presented as Acquired Thrombotic Thrombocytopenic Purpura: A Case Report.

Microangiopathic hemolytic anemia (MAHA) may occur as a paraneoplastic syndrome in some solid tumors, but MAHA accompanied by signet ring cell carcinoma (SRCC) of an unknown origin is very rare. We report a patient who presented with an acute onset of Coombs negative hemolytic anemia and frequent schistocytes in the peripheral blood smear which are typical for MAHA as initial presentation of metastatic SRCC. Our patients fulfilled the criteria of thrombotic thrombocytopenic purpura (TTP) and received the specific treatment for TTP without improvement.

Blastic Plasmacytoid Dendritic Cell Neoplasm: A Case Report and Clinicopathological Review.

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is aggressive hematopoietic malignancy derived from the precursors of plasmacytoid dendritic cells. The present study reported a case of a 35-year-old BPDCN patient, who presented with scalp lesions without extracutaneous involvement of the lymph nodes (LNs), peripheral or bone marrow. Histopathological examination of scalp lesion revealed monomorphous diffuse infiltrate of small to medium-sized cells with irregular nuclear contours, pleomorphic nuclei, finely dispersed chromatin, inconspicuous nucleoli and scant amount of cytoplasm. Immunohistochemical staining showed diffuse positivity for CD45, CD4, CD 56, CD45 and negative for CD3, CD5, CD7, CD8, CD19, CD20, CD30, CD33, CD34, CD79a, CD99, CD117, TDT, and myeloperoxidase. Patient started treatment with acute lymphoblastic lymphoma protocol (Hyper-CVAD). Reevaluation after the second course showed marked regression of scalp lesion. The patient continued Hyper-CVAD protocol and planned for allogeneic stem cell transplant.

Hypercalcemia With Disseminated Osteolytic Lesions: A Rare Presentation of Adulthood Acute Lymphoblastic Leukemia.

Osteolytic bone lesions and hypercalcemia without peripheral blasts B-cell acute lymphoblastic leukemia (B-ALL) are reported in children but rarely seen in adults. Herein, we described two patients with B-ALL presenting with hypercalcemia and symptomatic osteolytic bone lesions. They were treated by standard induction chemotherapy after correction of hypercalcemia with supportive measures. With this two case reports we would like to emphasize the importance of clinical awareness of hypercalcemia and osteolytic bone lesions as rare presentations of ALL. The prognostic implication of bone lesions and hypercalcemia in ALL is unclear and needs to be verified in large prospective studies. However, immediate recognition and treatment of hypercalcemia and the underlying B-ALL are vital since a delay of diagnosis poses a possible life-threatening risk.

Pregnancy associated acute leukemia: Single center experience.

INTRODUCTION: Data regarding clinical characteristics, therapy, maternal and fetal outcomes of pregnancy-associated acute leukemia are limited. PATIENTS AND METHODS: We herein, present the data of 27 pregnancy associated acute leukemia [18 acute myeloid leukemia (AML) and 9 acute lymphatic leukemia (ALL)]. Their data were compared to an age matched group of 75 non-pregnant acute leukemia patients admitted and treated during the same period [53 AML and 22 ALL]. RESULTS: Complete remission rates, induction failure and induction deaths showed insignificant differences in pregnant versus non pregnant patients in both types of acute leukemia. Similarly, OS and DFS showed no significant difference between pregnant versus non pregnant patients in both types of acute leukemia. Cox regression analysis was conducted for prediction of OS and DFS in all the studied patients applying age, WBCs, BM blasts, LDH, cytogenetic abnormalities and pregnancy as covariates, Pregnancy was not considered as a risk factor for OS or PFS in univariate or multivariate analysis. CONCLUSION: Our report to the best of our knowledge is the first to present direct evidence that pregnancy has no significant impact on outcome of acute leukemia compared to non-pregnant patients. These results are limited by the small number of cases and should be confirmed in a prospectively larger cohort of patients.