Myelophthisic anemia in cancer of the breast.
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Two cases of sarcoma complicated by pathologically confirmed myelophthisic anemia are presented. Although marrow involvement may occur in childhood rhabdomyosarcoma, complete marrow replacement in adult patients with sarcoma has not been previously described.
We report a 59-year-old, dark-complexioned black man with a giant basal cell carcinoma infiltrating virtually the entire scalp. Widespread metastatic bone marrow involvement produced a myelophthisic anemia. Basal cell carcinoma is rare in blacks, is rarely this large, and very rarely produces myelophthisic anemia from bone marrow metastases.
A 10-year-old, Golden Retriever-type castrated male dog was found to have a hemogram consistent with myelophthisic anemia. Within 30 days the hemogram changed in such a manner that a myeloproliferative disorder was evident. On the basis of hematologic, cytochemical, and cytologic findings, acute myelomonocytic leukemia was diagnosed.
The purpose of this study was to examine a mechanism which might explain the diminished erythropoiesis that occurs when tumor cells invade the bone marrow (myelophthisic anemia). To this end we compared 21 patients with neoplastic bone marrow invasion with seven normal subjects. When erythropoietin was added to bone marrow cell cultures from the normal subjects, the baseline rate of heme synthesis for a specific number of marrow erythroid cells increased 65%. In contrast, in patients with greater than 60% of the marrow replaced by tumor, the marrow cell culture response to erythropoietin was 14% above baseline. A significant inverse correlation was noted between the degree of marrow infiltration with tumor and the stimulation of heme synthesis with erythropoietin. It is proposed that marrow invasion with tumor may result in decreased marrow response to erythropoietin, and hence, diminished red cell production.
A case report of a metastasizing basal cell carcinoma is presented, primary in the leg with regional inguinal lymph node metastasis, followed by extensive metastasis to skin and bone marrow with myelophthisic anemia and terminal splenohepatic and unilateral adrenal metastasis.
A case report of liposarcoma occurring in a man with probable hereditary multiple lipomata and metastasizing to bone marrow is presented. The neoplasm was studied by light and electron microscopy. The first evidence for metastatic disease was myelophthisic anemia. This unusual clinical occurrence is discussed briefly in conjunction with other neoplasms causing myelophthisis.
In the typical patient with a metastasizing basal cell carcinoma, the tumor is large, ulcerated, and has been neglected. Recurrences are common, and the tumor is usually refractory to all modalities of treatment. Our patient neglected to seek medical help for 10 years, at which time metastases were already present. Our case is unique because the metastases to the skeletal system produced a myelophthisic anemia.
Accumulation of phagocytic histiocytes with a foam cell morphology has been described in a number of diseases. Familial hypercholesterolemia, one such disease, is characterized by foamy histiocytic accumulation in cutaneous or tendinous xanthomas and within atheromatous lesions. This report describes a patient with familial hypercholesterolemia who had two unusual manifestations of foamy histiocytic accumulation: a maxillary sinus xanthoma, which presented as an expansile mass, and diffuse bone marrow replacement with foamy histiocytes, which was associated with myelophthisic anemia. The accumulation of foamy cells in both locations resembled that seen in many of the storage diseases. The possibility of foamy histiocytic accumulation should be considered in the differential diagnosis of patients with these disease entities who present with space-occupying lesions.
Osteopetrosis is an inherited skeletal condition characterized by increased bone radiodensity. There are three clinical groups: infantile-malignant autosomal recessive, fatal within the first few years of life (in the absence of effective therapy); intermediate autosomal recessive, appears during the first decade of life but does not follow a malignant course; and autosomal dominant, with full-life expectancy but many orthopaedic problems. The infantile variant shows a myelophthisic anemia, granulocytopenia, and thrombocytopenia, and patients eventually die from infection or bleeding or both. Neurologic sequelae include cranial nerve compression (optic nerve, blindness; auditory nerve, deafness; facial nerve, paresis), hydrocephalus, convulsions, and mental retardation. Radiographs show uniform bone density without corticomedulary demarcation, broadened metaphyses, "bone within a bone" or endobone phenomena (tarsals, carpals, phalanges, vertebra, ilium), and thickened growth plates if there is superimposed rickets. Transverse pathologic fractures occur, often followed by massive periosteal bone formation. Computed tomographic scans, magnetic resonance imaging, and bone scans provide specific information. Iliac crest bone biopsy is valuable to quantitate osteoclast and marrow changes by light and electron microscopy. Medical treatments involve high-dose calcitriol to stimulate osteoclast differentiation and bone marrow transplantation to provide monocytic osteoclast precursors. Orthopaedic problems in the intermediate and autosomal dominant forms include increased fractures, coxa vara, long-bone bowing, hip and knee degenerative arthritis, and mandibular and long-bone osteomyelitis. Cranial nerve compression also occurs. Osteotomy, plating, intramedullary rodding, and joint arthroplasty can be done, but are difficult because of bone hardness.
We report a case of Kaposi's sarcoma of the bone marrow presenting as a myelophthisic anemia with bone marrow necrosis. The patient did not fit into any of the current clinical patterns described for Kaposi's sarcoma. This case illustrates the need for including Kaposi's sarcoma in the differential diagnosis of myelophthisic anemia.
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