Posted in Hematology, Hematopathology, Histopathology

Differences between Kimura disease and Angiolymphoid hyperplasia with eosinophilia

Kimura disease and Angiolymphoid hyperplasia with eosinophilia (ALHE) are often confused and need to be differentiated. Here are a few differences.

1.AGE

Kimura’s disease is a chronic inflammatory condition of unknown cause that affects young to middle-aged patients, most often males of Asian descent. Whereas, ALHE is more common in females.

2.LOCATION

Kimura disease patients usually have a mass in the head and neck region with involvement of subcutaneous tissue, soft tissue, salivary glands, and single or multiple regional lymph nodes. ALHE also commonly involves the head and neck region, particularly behind the ears.

3.HISTOLOGY

Key histologic features of kimura disease includes florid follicular hyperplasia that may contain a proteinaceous precipitate (IgE in a follicular dendritic network pattern) and vascularization of the germinal centers. The interfollicular areas show prominent high endothelial venules with a mixture of lymphocytes, plasma cells, eosinophils, and mast cells. Follicle lysis is often present, and eosinophilic abscesses are characteristic within germinal centers as well as in the paracortex.

ALHE on the other hand, is a vascular neoplasm characterized by the proliferation of blood vessels lined by plump endothelial cells with abundant eosinophilic cytoplasm, imparting a hobnail appearance. This lesion is part of the spectrum of what have been called histiocytoid or epithelioid hemangiomas, and is a low-grade vascular tumor. There is a dense, mixed inflammatory cell infiltrate consisting of lymphocytes, plasma cells, and eosinophils.

4. IMMUNOHISTOCHEMISTRY

Reticular pattern of IgE is seen in KIMURA disease. However, immunohistochemistry in ALHE is positive for CD 31, CD 34 and Factor VIII in the vascular component.

5.PERIPHERAL BLOOD EOSINOPHILIA

Peripheral blood examination shows eosinophilia and increased serum IgE levels in kimura disease but not so much in ALHE.

FIND A QUICK SUMMARY BELOW

REVIEW QUESTION

1. A 20- year- old Asian male has eosinophilia and high levels of IgE with cervical lymphadenopathy. Excisional biopsy of the lymph node demonstrates follicular hyperplasia, intense eosinophilia and eosinophilic microabscesses. h e most likely diagnosis is:

A. Kimura disease

B. Kikuchi- Fujimoto disease

C. Eosinophilic leukemia

D. Hypereosinophilic syndrome

CLICK TO REVEAL ANSWER

Answer is A- Kimura disease like Kikuchi disease is seen more ot en in the Asian population. h e etiology is not known. h is is a chronic inl ammatory disorder of the subcutaneous tissue and af ects regional lymph nodes. h e cervical area is the most common site to be involved. Histology of the af ected lymph nodes demonstrates follicular hyperplasia, eosinophilia with eosinophilic microabscesses and ini ltration of the germinal centers. Increase in vessels may also be seen. Warthin Finkeldey giant cells may also be present.

2. All are true about kimura disease except

A. Affects asians

B. Affects females

C. Peripheral eosinophilia is present

D. Elevated levels of IgE are seen

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Answer is B- Kimura disease is more common in males.

Posted in Hematology, Hematopathology

HEMOPHILIA B LEYDEN- A rare form of FIX deficiency

HEMOPHILIA B LEYDEN is a rare form of FIX deficiency

Here are some key points to be noted regarding Hemophilia B Leyden.

  1. It is a rare form of FIX deficiency, hemophilia B Leyden, undergoes postpubertal phenotypic resolution.
  2. Patients with this condition present with hemophilia B in early childhood, with FIX activity ranging from less than 1% to 13% of normal.
  3. Plasma levels rise to as high as 70% of normal after the onset of puberty with resolution of bleeding complications.
  4. Mutations in hemophilia B Leyden have been identified in the promoter region of the FIX gene, within which a consensus sequence for steroid receptor binding is located.

REVIEW QUESTION- A QUESTION FROM DM HEMATOPATHOLOGY PGIMER ENTRANCE

CLICK TO REVEAL THE ANSWER

ANSWER is C: Steroid receptor is involved in the pathogenesis.

Posted in Hematology, Hematopathology

MYH-9 associated congenital macrothrombocytopenias

MYH 9 associated congenital macrothrombocytopenias are important in an examination point of view. Let’s look at different types, key differences and some multiple choice questions.

A diverse group of autosomal dominant syndromes, referred to as MYH9-related macrothrombocytopenias, include the

  1. May-Hegglin anomaly,
  2. Fechtner syndrome,
  3. Epstein syndrome, and
  4. Sebastian syndrome

These share features of increased platelet size, cytoplasmic inclusions in leukocytes (Döhle bodies), and premature release of platelets. These entities arise from mutations in MYH9 encoding the nonmuscle myosin heavy chain IIA, a contractile cytoskeletal protein. All of the MYH-9 syndromes have macrothrombocytopenia; other features such as nephritis, sensorineural deafness, and cataracts serve to distinguish them.

FIND A QUICK DIFFERENTIAL DIAGNOSIS OF ALL MYH 9 ASSOCIATED DISORDERS

REVIEW QUESTIONS

1.A 19-year-old woman with a history of bruising and thrombocytopenia her entire life presents to a hematologist. Her review of systems is otherwise negative. Examination of the peripheral smear demonstrates platelets the size of the surrounding red blood cells (RBCs) and Döhle-like bodies in neutrophils. This presentation is most consistent with which platelet disorder?

A. Bernard Soulier syndrome.

B. May-Hegglin anomaly.

C. Fechtner syndrome.

D. Epstein syndrome.

CLICK HERE TO REVEAL ANSWER

Answer is B. May Hegglin anomaly presents with thrombocytopenia, giant platelets, and Döhle-like inclusions in neutrophils without hearing loss, nephritis, or cataracts.

2.A 20-year-old man with a history of bruising and thrombocytopenia her entire life presents to a hematologist. His review of systems revealed presence of nephritis, hearing loss and cataracts. Examination of the peripheral smear demonstrates platelets the size of the surrounding red blood cells (RBCs) and Döhle-like bodies in neutrophils. This presentation is most consistent with which platelet disorder?

A. Sebastian syndrome

B. Fechtner syndrome

C. Epstein syndrome

D. May Hegglin anomaly.

CLICK HERE TO REVEAL ANSWER

ANSWER: B: FECHTNER SYNDROME Characterized by thrombocytopenia, giant platelets, Döhle-like inclusions in neutrophils, hearing loss, nephritis, and cataracts.

Posted in Gastrointestinal pathology, Histopathology, WEEKLY QUIZZES

Gastrointestinal pathology MCQs

Gastrointestinal pathology MCQs – Useful for Pathology residents preparing for, NEET-SS/ DM- Oncopathology/ DM Histopathology, Fellowships, FRCPath- Histopathology and American Board of anatomic and clinical pathology (AP/CP) boards.

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