1/4. Cutaneous promyelocytic sarcoma at sites of vascular access and marrow aspiration. A characteristic localization of chloromas in acute promyelocytic leukemia?Extramedullary disease (EMD) is a rare clinical event in acute promyelocytic leukemia (APL). Although the skin is involved in half of the reported EMD cases, the occurrence of cutaneous promyelocytic sarcoma (PS) has been described very rarely. We report here three cases of PS which have the peculiarity of appearing at sites of punctures for arterial and venous blood and marrow samples (sternal manubrium, antecubital fossa, wrist over the radial artery pulse, catheter insertion scar). At presentation, all patients had hyperleukocytosis and a morphologic diagnosis of microgranular acute promyelocytic leukemia variant confirmed at the genetic level by demonstration of the specific chromosomal translocation t(15;17). A BCR3 type PML/RARa transcript was documented in the two patients for whom diagnostic RT-PCR was available. patients had morphologic bone marrow remission at the time the PS appeared. A predilection for the development of cutaneous PS at sites of previous vascular damage has been noted, but the pathogenesis remains largely unknown. A potential role for all-trans retinoic acid has been advocated, although one of the three patients in our series had received no ATRA. A review of the literature revealed six similar cases and hyperleukocytosis at diagnosis was a consistent finding in all of them. A careful physical examination of these particular sites in the follow-up of patients at risk, as well as cutaneous biopsy and laboratory examination of suspected lesions are strongly recommended.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
2/4. trismus in a 6 year old child: a manifestation of leukemia?trismus is a firm closing of the jaw due to tonic spasm of the muscles of mastication from disease or the motor branch of the trigeminal nerve. trismus may be produced by a variety of reasons such as dental abscess, trauma, following mandibular block with local anesthesia, as a result of radiation to the facial muscles, and patients after chemotherapy. A case of a referral of a six-year-old boy to a dentist from an ENT due to severe limitation in jaw opening is presented. Intraoral examination and panoramic radiograph demonstrated no signs of infection and/or other pathology. After a diagnosis of trismus was made, due to his icteric appearance, the general fatigue and loss of appetite in the last few days, palpated and sensitive lymph nodes in the submandibular and cervical regions, the child was referred for a complete blood count and sedimentation rate. The laboratory and clinical findings resulted in the diagnosis of acute lymphoblastic leukemia (ALL). Dental and oral manifestations of ALL are discussed, and the trismus may be explained by an intensive infiltration of leukemic cells into the deep portion of the contracting muscles of the face. This case emphasizes the importance of physical examination and independent judgement made by dentists, even when patients are referred to them by other members of the medical communities.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
3/4. Renal biopsy diagnosis of acute lymphocytic leukemia.hyperuricemia, due to inborn errors of metabolism, dehydration, or tumor lysis, may cause renal insufficiency. hyperuricemia from tumor lysis syndrome in malignancy is usually associated with electrolyte disturbances such as hyperkalemia, hyperphosphatemia or hyper or hypocalcemia. Tumor infiltration into the kidneys can occur, yet this accounts for renal insufficiency in only 1% of patients. This infiltration of tumor cells into the kidneys is usually associated with evidence of malignancy elsewhere as identified by physical exam, radiographic studies, and examination of the peripheral smear or bone marrow. We report an unusual presentation of a child with acute lymphocytic leukemia presenting with acute renal failure, nephromegaly and hyperuricemia without electrolyte disturbances or systemic evidence of tumor elsewhere. We stress the importance of kidney biopsy in order to identify the etiology of the renal failure and hyperuricemia.- - - - - - - - - - ranking = 0.04438872613677keywords = physical (Clic here for more details about this article) |
4/4. T-cell lymphoblastic lymphoma presenting with a breast mass.Lymphomas secondarily involving the breast are uncommon, although they do represent the largest group of tumors metastatic to breast. A 20-year-old female with lymphoblastic lymphoma (LBL) presented here with 3 month history of weight loss, night sweats, fatigue and a mass in her left breast. Her physical examination revealed a left breast mass, lympadenopathy, bilateral pleural effusion and hepatomegaly. WBC count was 17,710/mm3 and LDH was mildly elevated. breast ultrasound showed a 1.7 cm mass in the inner lower quadrant of left breast. biopsy of the breast mass showed diffuse infiltration with small, round atypical cells which did not stain with CD20, CD43, CD34, cytokeratine and were positive for CD3. She was diagnosed as leukemic phase of a precursor T-cell LBL and treated with 6 cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone), intrathecal methotrexate and cranial radiotherapy, achieving a complete response. She then was started on maintenance therapy. Four months later she returned with CNS involvement and was started on induction treatment. She had a very aggressive course of disease and died only 12 months after diagnosis. breast involvement is very rarely seen in precursor T-cell LBL/ALL and in this patient occurred secondarily as part of widespread disease.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |