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Langerhans cell histiocytosis presenting as blueberry muffin baby

Blueberry muffin baby is a descriptive term for purpuric lesions reflective of extramedullary hematopoeisis. The clinical lesions most commonly result from intrauterine infections, such as rubella and cytomegalovirus, and less commonly with malignancy and hematologic disorders. Langerhans cell histiocytosis is a clonal proliferation of dendritic histiocytes in the skin Langerhans cell histiocytosis presenting as a blueberry muffin rash. A baby boy born at term via spontaneous vaginal delivery to a healthy mother was noted to have a widely disseminated blueberry muffin rash at birth. The pregnancy had been uncomplicated and the patient was otherwise healthy—he was active, afebrile, and feeding well At the pediatric dermatology unit of our clinic, during the last 20 years, we had 10 children with Langerhans cell histiocytosis and among them only one with CSHLCH. In the literature, we found only 5 newborns with Langerhans cell histiocytosis presenting as blueberry muffin baby, among them only 4 with self-healing CSHLCH In our case report, we discuss a 1-day-old boy presenting with blueberry muffin syndrome diagnosed with Langerhans cell histiocytosis. The diagnosis complicated by an initial difficult-to-interpret biopsy showing only a hint of perifollicular CD1a-positive cells; however, given our team's strong clinical suspicion of Langerhans cell histiocytosis, a second biopsy of a more mature lesion was.

Langerhans cell histiocytosis presenting as a blueberry

  1. Langerhans cell histiocytosis presenting as blueberry muffin baby. Shaffer MP , Walling HW , Stone MS J Am Acad Dermatol , 53(2 suppl 1):S143-6, 01 Aug 200
  2. The tumors such as Langherhans cell histiocytosis, Neuroblastoma, Leukaemia cutis and Rhabdomyosarcoma are also an important cause for the Blue berry muffin baby syndrome. Langherhans cell histiocytosis involves the clonal proliferation of Langerhans cells, abnormal cells derivin
  3. 3. Shaffer MP, Walling HW, Stone MS. Langerhans cell histiocytosis presenting as blueberry muffin baby. J Am Acad Dermatol. 2005;53:S143-S146. 4. Baselga E, Drolet BA, Esterly NB. Purpura in infants and children. J am acad Dermatol. 1997;37:673-707. 5. Taj FT, Sarin V. Blueberry muffin baby (dermal erythropoiesis) with non-ketotic hyperglycinemia
  4. Blueberry muffin baby is the characteristic distributed purpura occurring as a result of extramedullary hematopoiesis found in infants.: 826 The purpura are often generalized, but occur more often on the trunk, head, and neck. The name is from the superficial similarity to a blueberry muffin
  5. Blueberry muffin baby: A pictoral differential diagnosisVandana Mehta, C Balachandran, Vrushali LonikarDermatology Online Journal 14 (2): 8. The term blueberry muffin baby was initially coined by pediatricians to describe cutaneous manifestations observed in newborns infected with rubella during the American epidemic of the 1960s [ 1 ]

Langerhans cell histiocytosis. Contributors. All authors contributed to patient management and writing of the report. Written consent to publication was obtained from the parents. Langerhans cell histiocytosis presenting as a blueberry muffin rash. Adam R Schmitt, David A Wetter, Michael J Camilleri, Shakila P Khan, Megha M Tollefson. Lancet. langerhan cell histiocytosis, neuroblastoma and congenital leukemia1 •We report a rare case of congenital non-langerhan cell histiocytosis (NLCH) presenting as blueberry muffin baby INTRODUCTION CASE DESCRIPTION •A 25 day old newborn girl born at term to healthy Indian parents presented with purpuric rash for the past 10 day

Shaffer MP, Walling HW, Stone MS. Langerhans cell histiocytosis presenting as blueberry muffin baby. J Am Acad Dermatol. 2005 Aug. 53(2 Suppl 1):S143-6. . Hwang C, Kim YJ, Seo YJ, Park JK, Lee JH, Lee Y. Isolated langerhans cell histiocytosis of the vulva in an infant. Pediatr Dermatol. 2009 Nov-Dec. 26(6):751-3. Shaffer MP, Walling HW, Stone MS. Langerhans cell histiocytosis presenting as blueberry muffin baby. J Am Acad Dermatol . 2005 Aug. 53(2 Suppl 1):S143-6. [Medline] Shaffer MP, Walling HW, Stone MS.Langerhans cell histiocytosis presenting as blueberry muffin baby. J Am Acad Dermatol. 2005 Aug;53(2 Suppl 1):S143-6. Braier J, Chantada G, Rosso D, Bernaldez P, Amaral D, Latella A, Balancini B, Masautis A, Goldberg J. Langerhans cell histiocytosis: retrospective evaluation of 123 patients at a single institution Langerhans cell histiocytosis is a rare condition that can affect any organ of the body. Patients of all ages may present to the dermatologist and it is important to make the diagnosis as quickly as possible, because time from presentation to diagnosis is of prognostic importance in adults with the disease Blueberry muffin syndrome (a.k.a. blueberry muffin baby or rash) refers to a skin appearance seen in paediatric patients with multiple raised cutaneous, classically blue/purple lesions which is due to extramedullary haematopoiesis in the dermis. The syndrome was originally described in congenital rubella cases in the United States in the 1960s 3

Congenital Self-Healing Histiocytosis Presenting as

Langerhans cell histiocytosis presenting as blueberry muffin baby MatthewP.Shaffer,MD,a Hobart W. Walling, MD, PhD,a andMarySeaburyStone,MDa,b Iowa City, Iowa Blueberry muffin baby is a descriptive term for purpuric lesions reflective of extramedullary hematopoeisis Although cases of neonatal LCH presenting as a blueberry muffin baby or mimicking neonatal hemangiomatosis have been reported, , a combination of diffuse hemorrhagic nodules and a giant cutaneous mass has not been published. Due to elevation of LDH, lung and cutaneous involvement, our case can be classified as disseminated LCH Blueberry-Muffin rash, Infant, Langerhans cell histiocytosis. Introduction. Langerhan cell tissue hyperplasia (langerhans cell histiocytosis, LCH) caused by the hyperplasia of langerhans cell is rare and the incidence is 1-2/1000, 000 . The clinical manifestations are diversification involving skin, bone, lymph nodes, liver, spleen, lung.

Keywords: Blueberry-Muffin rash, Infant, Langerhans cell histiocytosis. Accepted on December 19, 2016 Introduction Langerhan cell tissue hyperplasia (langerhans cell histiocytosis, LCH) caused by the hyperplasia of langerhans cell is rare and the incidence is 1-2/1000, 000 [1]. The clinica Detailed evaluation of a baby with blueberry muffin lesions is needed since it is an initial presenting feature of various aetiologies. Langerhans cell histiocytosis, Hashimato-Pritzker disease (congenital self-healing reticulohistiocytosis), lupus. Langerhans cell histiocytosis [LCH], neuroblastoma), and skin disorders (hemangiomatosis, blue rubber bleb nevus syndrome). Cytomegalovirus (CMV) is the most common congenital infection and can cause a petechial rash due to thrombocytopenia. Congenital toxoplasmosis has been described with this type of rash, but it is a rar Keywords: Juvenile xanthogranuloma, Non-Langerhans cell histiocytosis, Blueberry muffin baby, Case report, Systemic, Histopathology Background Juvenile xanthogranuloma (JXG) is a rare 'histiocytic' disorder and belongs to the broad group of non-Langerhans cell histiocytosis [1]. As noted in a re-port of this condition by Helwig and Hackney. A neonate with Langerhans cell histiocytosis presenting as blueberry muffin rash: Case report and review of the literature Janelle Cyr1, Annie Langley2, Dina El Demellawy3 and Michele Ramien2,4 Abstract In our case report, we discuss a 1-day-old boy presenting with blueberry muffin syndrome diagnosed with Langerhans cell histiocytosis

Hemorrhagic pustules with a blueberry muffin appearance accompanied by respiratory failure in a neonate present a challenging differential diagnosis that includes infections and neoplasms. We present a case of multiorgan, multisite Langerhans cell histiocytosis (LCH), positive for the oncogenic BRAF V600E mutation, in a preterm neonate. Langerhans cell histiocytosis presenting as blueberry muffin baby Shaffer MP, Walling HW, Stone MS. J Am Acad Dermatol.2005;53(2 Suppl 1):S143-S146. CrossRef; Structural correlations between dermoscopic and histopathological features of juvenile xanthogranuloma Song M, Kim SH, Jung DS, Ko HC, Kwon KS, Kim MB Shaffer MP, Walling HW, Stone MS. Langerhans cell histiocytosis presenting as blueberry muffin baby. J Am Acad Dermatol. 2005;53(2 Suppl 1):S143-6. Article PubMed Google Scholar 7. Hebert AA, Esterly NB, Gardner TH. Dermal erythropoiesis in Rh hemolytic disease of the newborn. J Pediatr. 1985;107(5):799-801 ~ By a retired Pediatric Hematologist Oncologist in Canada. A recent patient case on Sermo reminded me of seeing an 11-day-old baby with reddish-blue skin lesions and mild hepatomegaly.. This presentation reminded me of a Blueberry Muffin Baby for which there was a whole slew of differential diagnosis, including the TORCH infections (toxoplasmosis, other, rubella, cytomegalovirus, herpes. Tucker C: Langerhans cell histiocytosis presenting as a blueberry muffin baby. J Am Acad Dermatol. 2013; 68(4): AB175. Publisher Full Text ; 55. Tabassum F, Debelenko L, Savasan S: 19 month old male with clinical diagnosis of congenital lamellar Ichthyosis and multiorgan involvement by langerhans cell histiocytosis. 2011. 56

CSHR is a rare benign variant of Langerhans cell histiocytosis which is generally confined to the skin and is self-limited.1 Typical cutaneous manifestations in neonates include purplish to violaceous-reddish vesiculopustules and papulonodules.2 Differential diagnoses of this condition a rare presentation of blueberry muffin baby spectrum Main Pediatric Blood & Cancer Diverse Cutaneous Presentations of Langerhans Cell Histiocytosis in Children: A Retrospective Cohort.. Pediatric Blood & Cancer 2016 / 03 Vol. 63; Iss. 3. Diverse Cutaneous Presentations of Langerhans Cell Histiocytosis in Children: A Retrospective Cohort Stud A newborn baby with generalized blueberry muffin eruption and histologic evidence of dermal erythropoiesis is described. This case is unusual in that we could not identify any of the congenital viral infections usually associated with this syndrome. The clinical features of dermal erythropoiesis and dermal hematopoiesis are reviewed briefly Langerhans cell histiocytosis presenting as blueberry muffin baby Matthew P. Shaffer, Hobart W. Walling, Mary Seabury Stone Glutaraldehyde-induced and formaldehyde-induced allergic contact. Blueberry muffin baby is the term given to characteristic eruption in neonates, often present at birth, comprising widespread, erythematous, purple, oval or circular macules, papules and nodules reflecting dermal erythropoiesis seen in a number of congenital infections, notably rubella, cytomegalovirus, coxsackie B2 infection, parvovirus B19, congenital syphilis, toxoplasmosis, Rh.

14. Munn S, Chu AC. Langerhans cell histiocytosis of the skin. Hematol Oncol Clin North Am. 1998;12:269-286. 15. Popadic S, Brasanac D, Arsov B, Nikolic M. Congenital self-healing histiocytosis presenting as blueberry muffin baby: a case report and literature review. Indian J Dermatol Venereol Leprol. 2012;78:407. 16 Blueberry muffin syndrome (a.k.a. blueberry muffin baby or rash) refers to a skin appearance seen in pediatric patients with multiple raised cutaneous, classically blue/purple lesions which is due to extramedullary hematopoiesis in the dermis. The syndrome was originally described in congenital rubella cases in the United States in the 1960s 3 Langerhans cell histiocytosis (LCH) is a clonal disorder that is characterized by proliferation of Langerhans cell in various organs of the body. The annual incidence rate is 0.5-5.4 million children per year. Congenital self-healing reticulohistiocytosis (CSHR) is considered to be at one end of the spectrum of LCH Congenital Langerhans cell histiocytosis (LCH) is a rare condition with great diversity. A case of c. Log in. Your account has been temporarily locked. Your account has been temporarily locked due to incorrect sign in attempts and will be automatically unlocked in 30 mins..

2 Hansel K, Tramontana M, Troiani S, et al. Congenital self-healing langerhans cell histiocytosis: a rare presentation of blueberry muffin baby spectrum. Dermatopathology 2019;6:37-40. 3 Avram MM, Gobel V, Sepehr A. Case records of the Massachusetts General Hospital. Case 30-2007. A newborn girl with skin lesions. N Engl J Med 2007;357:1327. histiocytosis non langerhans cell. Wikipedia. Medical Information Search. English. English Español Português Français Italiano Svenska Deutsch Langerhans cell histiocytosis presenting as blueberry muffin baby. J. Am. Acad. Dermatol. 53 (2 Suppl 1): S143-6. doi:10.1016/.

A neonate with Langerhans cell histiocytosis presenting as

Blueberry muffin baby is the characteristic distributed purpura occurring as a result of extramedullary hematopoiesis found in infants.:826 The purpura are often generalized, but occur more often on the trunk, head, and neck. The name is from the superficial similarity to a blueberry muffin Congenital self-healing reticulohistiocytosis (CSHRH) is a rare type of Langerhans cell histiocytosis with potential for relapse and systemic involvement. Whereas CSHRH was traditionally considered a benign disease, there is an approximately 3 percent risk of mortality and a 10 percent chance of relapse Langerhans cell histiocytosis presenting in the neonatal period: a retrospective case series. Stein SL, Paller AS, Haut PR, Mancini AJ. Department of Dermatology, Northwestern University Medical School, Chicago, IL 60614, USA. Blueberry muffin baby is a descriptive term for purpuric lesions reflective of extramedullary hematopoeisis. The. Langerhans cell histiocytosis. Tony Chu. Head of Section of Dermatology, Imperial College of Science, Technology and Medicine, Hammersmith Campus, London, United Kingdom. Search for more papers by this author. 8. Popadic S, Brasanac D, Arsov B, Nikolic M. Congenital self-healing histiocytosis presenting as blueberry muffin baby: a case report and literature review. Indian J Dermatol Venereol Leprol. 2012;78:407

Indian Journal of Dermatology, Venereology and Leprology

Langerhans cell histiocytosis presenting as blueberry

6 Butler DF, Ranatunge BD, Rapini RP. Urticating Hashimoto-Pritzker Langerhans cell histiocytosis. Pediatr Dermatol. 2001;18:41-4. [ Links ] 7 Shaffer MP, Walling HW, Stone MS. Langerhans cell histiocytosis presenting as blueberry muffin baby. J Am Acad Dermatol. 2005;53:S143-6. [ Links ] 8 Lee YH, Talekar MK, Chung CG, Bell MD, Zaenglein AL Blueberry muffin baby (225 words) exact match in snippet view article find links to article cutaneous conditions Shaffer MP, Walling HW, Stone MS (August 2005). Langerhans cell histiocytosis presenting as blueberry muffin baby. J. Am. Acad. Dermato Langerhans cell histiocytosis is a rare condition, slightly more common in boys than girls, affecting around one in 5 million children and fewer adults. Langerhans cell histiocytosis may develop at any age but most commonly occurs in childhood (1-3 years of age) Langerhans cell histiocytosis (LCH) is the main .2010.191296 2-s2.0-78349291118 76 Kitagawa N. Arata J. Ohtsuki Y. Hayashi K. Oomori Y. Tomoda T. Congenital alveolar rhabdomyosarcoma presenting as a blueberry muffin baby Journal of Dermatology 1989 16 5 409 411 10.1111/j.1346-8138.1989.tb01291.x 2-s2.0-0024431341 77 Sultan I. Casanova M. Al. Langerhans cell histiocytosis (LCH) is the main histiocytosis occurring in the neonatal period, Y. Oomori, and T. Tomoda, Congenital alveolar rhabdomyosarcoma presenting as a blueberry muffin baby, Journal of Dermatology, vol. 16, no. 5, pp. 409-411, 1989. View at: Publisher Site | Google Scholar

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[Blueberry muffin baby]

Blueberry Muffin Baby is a rare neonatal cutaneous syndrome for purpuric lesions reflective of extramedullary hematopoiesis. Many causes are known, examples are congenital infections, malignancy and hematologic disorders. Langerhans' cell histiocytosis is a clonal proliferation of dendritic histiocytes healing histiocytosis presenting as blueberry muffin baby: a case report and literature review. Indian J Dermatol Venereol Leprol. 2012;78:407. 9. Hoeger PH, Nanduri VR, Harper JI, Atherton DA, Pritchard J. Long term follow up of topical mustine treatment for cutaneous langerhans cell histiocytosis. Arch Dis Child. 2000;82:483-487. 10 •Present at birth and resolves within 24-48 hours Blueberry Muffin -Langerhans cell histiocytosis From Box 15-3 Neonatal Dermatology, ed. Eichenfeld, Frieden & Esterly. Empiric Treatment - Afebrile, eating, stooling, activity @ baseline Home care: - Dilute bleach baths dail

Blueberry Muffin Baby Syndrome (BMB) treatment and errors

Blueberry muffin baby - Wikipedi

Blueberry muffin baby: A pictoral differential diagnosi

Langerhans cell histiocytosis presenting as blueberry muffin baby Matthew P. Shaffer, Hobart W. Walling, Mary Seabury Stone> ;Journal of The American Academy of Dermatology. 2005 Aug 1 11 citation Congenital Langherhans cell histiocytosis (LCH) is a rare immune disorder, presenting during the neonatal period with variable cutaneous finding and sometimes as disseminated disease. We describe a case of LCH in large for gestational age (LGA) term male neonate, who presented with the single face skin lesion

Langerhans' cell histiocytosis. Langerhans' cell histiocytosis (LCH), a rare proliferative disorder, may be present at birth or may develop during the first few months of life. Presentation as a 'blueberry muffin baby,' with cutaneous hematopoiesis, has been reported. 24 Nodules and petechiae, which may involve the palms and soles. Langerhans cell histiocytosis; Bullous mastocytosis (Figure 8) Histology Plus giemsa/toluidine blue: Scattered bluish nodules and papules are seen in the 'blueberry muffin baby' and may represent an underlying tumour (eg. neuroblastoma, rhabdomyosarcoma, leukaemia), infection (toxoplasmosis, rubella, cytomegalovirus, coxsackie B2.

Blueberry Muffin Rash- . The blueberry muffin rash is aptly named as this condition has the characteristics of a blueberry muffin.. The skin exhibits blue-grey nodules or a purpuric rash. It appears on infants and requires immediate consultation with the pediatric dermatology department and infectious disease specialists, oncologists, pediatricians and neonatologists Langerhans cell histiocytosis (LCH), previously known as Histiocytosis X, is a condition characterized by a clonal proliferation of CD1a+ LC. LCH is the type of histiocytosis observed with higher frequency [5, 11].Clinical presentation and prognosis vary depending on the site and number of organs affected

Langerhans Cell Histiocytosis Clinical Presentation

Langerhans' cell histiocytosis (LCH), a rare proliferative disorder, may be present at birth or may develop during the first few months of life. The estimated incidence of neonatal LCH is 9/1 000 000 in infants <1 year of age, with disease presentation during the first month of life in about 6% of those affected Langerhans cells were present in the dermis that stained CD1a positive, S-100 positive, and CD68 negative to con - firm the diagnosis. Congenital self-healing reticulohistiocytosis, or Hashimoto-Pritzker syndrome, is a rare benign form of . Langerhans cell histiocytosis. It is twice as common in males than females and typically noted at birth. Langerhans cell histiocytosis: a review of the non-Langerhans cell histiocytoses (class II), and malignant histiocytosis (class III). Langerhans cell histiocytosis presenting as blueberry muffin baby. J. Am. Acad. Dermatol. 53 (2 Suppl 1): S143-6. doi:10.1016/. As a clonal proliferation of Langerhans cells, pathology reveals lesions that stain positive for CD1a and S-100. 2. Although typically absent, evaluation for systemic involvement is warranted, which may be an early presentation of multisystem Langerhans cell histiocytosis Abstract Background Juvenile xanthogranuloma (JXG) belongs to the heterogeneous group of non-Langerhans cell histiocytosis and is caused by an accumulation and proliferation of macrophages. In the majority of cases JXG is a disorder of early childhood presenting during the first 2 years of life

Langerhans cell hyperplasia of the skin mimicking Langerhans cell histiocytosis: A report of two cases in children not associated with scabies. Fetal Pediatr. Pathol. 2010 ; 29 (4): 231 -8 Blueberry muffin rash and large rightm thigh mass: a unique presentation of Langerhans cell histiocytosis. Dermatol Online J, 2013 Jun 15;19(6):18568. Brittain P, Walsh, EJ, Smidt AC. Blotchy baby: a case of phakomatosis pigmentovascularis. J Pediatr. 2013 Jun;162(6):1293. Berry T, Shetty A, Delu A, Barry M, Berry R, Smidt AC. Presternal. Langerhans cell histiocytosis presenting as blueberry muffin baby. Drug-induced delusions of parasitosis during treatment of Parkinson's disease. Basal cell carcinoma responding to systemic 5-fluorouracil. Benign cutaneous Degos disease in a 16-year-old girl. Papular eruption in an HIV-infected man with Langerhans cell histiocytosis (LCH). Histiocytoses are a heterogeneous group of disorders . in which the infiltrating cells belong to the mononuclear phagocyte system. 1,2. Langerhans cell histiocytosis is the most common dendritic cell-related histiocytosis, occur-ring in approximately 5 per 1 million children annually

അസ്ഥിമജ്ജയ്ക്ക് പുറമേ ഉള്ള അവയവങ്ങളിൽ രക്തകോശങ്ങളുടെ. Collodion membrane is most closely associated with forms of autosomal recessive congenital ichthyosis, but the differential diagnosis includes many other less common etiologies. Herein we present a case of Gaucher disease (GD) type 2 in a neonate presenting with collodion membrane in addition to blueberry muffin lesions. The clinical presentation and etiology of GD and the differential. The clinical presentation consists of erythematous to violaceous papules and nodules which may ulcerate. When widely disseminated in neonates, it leads to the classic blueberry muffin baby ( Fig. 121.6 ) . This can be seen in association with congenital viral infections and prenatal anemias ( Table 121.4 ). Dermal hematopoiesis is most. Neonatal leukaemia cutis is a significant neoplasm that may represent a cutaneous manifestation of systemic leukaemia, usually of myeloblastic type. Rarely, it may be or appear to be limited to skin, in which case it is called neonatal aleukaemic leukaemia cutis. By definition, it presents within the first 4 weeks of life and often has a 'blueberry muffin baby' appearance of magenta.

Which histologic findings are characteristic of Langerhans

cases. Leukemia cutis, presenting as firm nodules, pap-ules or plaques of red, blue or purple color, is a differen-tial diagnosis of the so-called blueberry muffin baby. This syndrome has a diversified differential, ranging from hemato-oncological disorders like Langerhans cell histiocytosis, neuroblastoma, or rhabdomyosarcoma to in Other conditions included in the differential are autoimmune conditions (purpuric variant of neonatal lupus), hematologic conditions (blood group incompatibility, twin-to-twin transfusion syndrome), skin conditions (blue rubber bleb nevus syndrome, hemangiomatosis), and other types of malignancies (Langerhans cell histiocytosis, neuroblastoma). 4,

2015, Lai-San Wong, An Unusual Case of Neonatal Langerhans Cell Histiocytosis Presenting with Diffuse Hemorrhagic Nodules and a Cutaneous Abdominal Mass, in Indian Journal of Dermatology‎, volume 60, DOI: 10.4103/0019-5154.160545 Chronic multifocal Langerhans cell histiocytosis, previously known as Hand-Schüller-Christian disease, is a type of Langerhans cell histiocytosis (LCH), which can affect multiple organs.The condition is traditionally associated with a combination of three features; bulging eyes, breakdown of bone, and diabetes insipidus, although around 75% of cases do not have all three features Pustules or hyper-pigmented macules with collarette of scale present at birth Smear with NEUTROPHILS subcorneal pustules with neutrophils. Erythema Toxicum Neonatorum (ETN) caucasian babies full term 50% (rare in preterm, low birthweight) 1-2 days after birth with blotchy pink papules, pustules, wheal Histiocytosis is a tumor-like condition, frequently seen in children between 1 and 15 years. However, the etiology is poorly defined. One hypothesis suggests that it may be caused by an alteration of the Langerhans cell by a somatic mutation or a viral infection at an early stage of develop-ment .The cell accumulates abnormally an Mucolipidosis type II (I—cell disease) Neurodevelopmental disorder Aicardi-Goutières syndrome Learning points 1. Detailed evaluation of a baby with blueberry muffin lesions is needed since it is an initial presenting feature of various aetiologies. 2. Extramedullary haematopoiesis should be differentiated from chloroma, which is usually.