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Through humoral and cell-mediated mechanisms, the immune system plays a vital role in protecting every organ system. Disorders of the immune system may result in various cutaneous manifestations, including cutaneous malignancies. In patients with immunodeficiency, the risk of development of malignant cutaneous neoplasms is substantially increased. This increased risk may be due to oncogenic viruses that find a suitable microenvironment for tumorigenesis and cancer development. A subset of cutaneous malignancies that develop in patients with immunodeficiency may show aggressive clinical and biological behavior. Here, we report six cases of highly aggressive and deadly cutaneous neoplasms that arose in patients with a known history of immunodeficiency: two cases of Kaposi sarcoma in patients with immunosuppression due to human immunodeficiency virus infection; a case of Merkel cell carcinoma and a case of squamous cell carcinoma (SCC) in patients receiving immunosuppressive drugs after organ transplant; a case of multiple cutaneous tumors, including invasive melanoma, SCC, and sebaceous carcinoma, in a patient with hypogammaglobulinemia and a history of organ transplant; and a case of basal cell carcinoma and melanoma in situ in a patient with primary immunodeficiency.

Context.—The cause of pancreatic acinar metaplasia (PAM) at the distal esophagus/esophagogastric junction is still controversial. Whereas some authors believe it is congenital, others believe it is acquired because of inflammation of the gastric cardia, and more recently it was proposed to be due to chronic proton pump inhibitor use based on a study in rats.Objective.—To determine whether there is correlation between chronic proton pump inhibitor use and PAM in humans. We also investigated the correlation between several clinical and pathologic factors and PAM.Design.—Four hundred forty-four consecutive biopsies from the distal esophagus/esophagogastric junction were reviewed for the presence of PAM, which was then correlated with several clinical and pathologic findings.Results.—Pancreatic acinar metaplasia was found in 71 patients (16%). Pancreatic acinar metaplasia was significantly associated with patient age younger than 51 years (P < .001), chronic carditis (P = .01), and chronic proton pump inhibitor use (P = .008). Surprisingly, we also found significant association between PAM and chronic nonsteroidal anti-inflammatory drug use (P < .001). These associations, including that with chronic nonsteroidal anti-inflammatory drug use, remained significant in multivariate analysis.Conclusions.—Our findings confirm the previous reports of significant association between PAM and chronic carditis and the findings from animal studies of association with chronic proton pump inhibitor use. The strong association with chronic nonsteroidal anti-inflammatory drug use has not been previously reported and warrants further studies.

Most of metastatic tumors to the skin are from primary tumors of the breast, lung, or from melanoma; metastases to the skin from primary carcinomas at other sites are rare. Cutaneous metastases of visceral carcinomas most often occur in patients with advanced disease, and are associated with a poor prognosis. We report 6 cases of nonmammary, nonpulmonary carcinoma metastatic to the skin. Most patients were elderly with advanced disease at the time of diagnosis of skin metastasis. The primary tumor sites included the thyroid, esophagus, biliary tract, ovary, and prostate. Awareness of these rare cases of metastasis to the skin will help pathologists and clinicians make the correct diagnosis.

The action of bacterial neuraminidase ofA 6-month-old boy presented with respiratory failure. He was found to have blood cultures positive for SPN. Shiga toxin was negative and he had normal levels of ADAMTS 13 (a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13). The clinical team was concerned for pHUS and requested washed platelet product prior to a surgical procedure. Alternatively, a minor crossmatching was performed to determine the presence of T activation. An aHUS genetic panel was performed to sequence and analyze 12 genes encoding complement factors.Minor crossmatch was performed using the patient's erythrocytes and plasma of ABO-identical platelets to be transfused. No agglutination was seen at immediate spin, 37°C, or anti-human globulin phase with valid controls. Genetics testing for complement mutations was consistent with aHUS.We present a case that is clinically consistent with pHUS. Confirmation of this entity is done with lectins or anti-sera that are not readily available. An alternative means of identifying pHUS is by demonstrating minor crossmatch incompatibility. By doing so, we excluded the possibility of pHUS and helped to elucidate a definitive diagnosis of aHUS. Our goal is to share our experience of a practical approach in a time-sensitive situation that other clinical pathologists could utilize in suspected cases of T activation with a clinical picture of thrombotic microangiopathy.

Post-transplant lymphoproliferative disorders (PTLD) are emergent complications of organ transplantation occurring in 2% to 10% of transplanted patients. Epstein-Barr virus (EBV) infections are considered the most important factors for the development of these heterogeneous disorders. Primary effusion lymphoma (PEL) is a lymphoproliferative disorder predominantly described in patients with advanced AIDS and it is almost universally associated with human herpesvirus 8 (HHV8). In rare case, PEL also occurs in HHV8-negative patient, in the setting of hepatitis B and C virus infection. However, all these cases showed pan B-cell markers to be positive. Here, we report a case of PTLD presented as HHV8-negative and HIV-negative primary effusion lymphoma lacking near all lymphoid markers except PAX5 on immunohistochemistry, which created a diagnostic challenge. The diagnosis requires multiple approaches including molecular and genetic tests.

A morphoproteomic analysis was performed to identify the proteins and corresponding molecular pathways activated for a case of non-HIV Kaposi's sarcoma. This analysis provides insight into the biology of the tumor and identifies etiopathogenetic correlates of HHV-8-Associated Kaposi's sarcoma that are useful in formulating therapeutic alternatives.

TO THE EDITOR: Hemophagocytic lymphohistiocytosis (HLH) is characterized by severe immune activation and deregulation resulting in extreme and often life-threatening inflammation [1]. Adult-onset HLH is rare and fatal. Infectious agents contribute to a major part of the etiology of adult-onset HLH [2,3]. A high degree of clinical suspicion and prompt treatment is required to prevent mortality. We have described an unusual clinical presentation of a case of cytomegalovirus (CMV)-associated HLH with multi-organ involvement.